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Hancıoğlu E, Özcan S, Dönmez E, Terzioğlu C, Kırankaya A, Tenekecigil A, Okuyan E, Sahin İ. The relationship between serum Lumican levels and myocardial fibrosis in patients with hypertrophic cardiomyopathy. Biomark Med 2025; 19:187-195. [PMID: 40033847 PMCID: PMC11916418 DOI: 10.1080/17520363.2025.2473304] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/14/2024] [Accepted: 02/25/2025] [Indexed: 03/05/2025] Open
Abstract
OBJECTIVE Hypertrophic cardiomyopathy (HCM) is the leading cause of sudden cardiac death in young individuals. Lumican, is an indicator of fibrosis. We aimed to evaluate the relation between serum Lumican levels and presence and extent of myocardial fibrosis in HCM. METHODS The patients diagnosed with HCM between June 2022 and March 2023 were enrolled consecutively in this prospective study. Age and gender-matched healthy individuals formed control group. Two groups were generated according to extent of late gadolinium enhancement (LGE) in HCM patients. RESULTS A total of 114 patients were enrolled. Serum Lumican, NT-proBNP levels and maximum wall thickness were revealed as independent risk factors associated with LGE. A cut-off value of 9.06 for Lumican was associated with 67.8% sensitivity and 65.5% specificity in prediction of LGE. CONCLUSION Myocardial fibrosis is one of the important mechanisms in HCM pathophysiology. LGE on cardiac magnetic resonance imaging allows comprehensive evaluation of myocardial fibrosis, the support of diagnosis with a biomarker would be beneficial in clinical practice. Our study revealed that serum Lumican level is an independent predictor of severe LGE in HCM patients. Further studies with larger patient numbers may clarify the importance of Lumican in HCM pathophysiology.
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Affiliation(s)
- Emirhan Hancıoğlu
- Department of Cardiology, Bağcılar Training and Research Hospital, Bağcılar, İstanbul, Turkey
| | - Sevgi Özcan
- Department of Cardiology, Bağcılar Training and Research Hospital, Bağcılar, İstanbul, Turkey
| | - Esra Dönmez
- Department of Cardiology, Bağcılar Training and Research Hospital, Bağcılar, İstanbul, Turkey
| | - Cemal Terzioğlu
- Department of Cardiology, Bağcılar Training and Research Hospital, Bağcılar, İstanbul, Turkey
| | - Ayşegül Kırankaya
- Department of Biochemistry, Bağcılar Training and Research Hospital, Bağcılar, İstanbul, Turkey
| | - Aslıhan Tenekecigil
- Department of Biochemistry, Bağcılar Training and Research Hospital, Bağcılar, İstanbul, Turkey
| | - Ertugrul Okuyan
- Department of Cardiology, Bağcılar Training and Research Hospital, Bağcılar, İstanbul, Turkey
| | - İrfan Sahin
- Department of Cardiology, Bağcılar Training and Research Hospital, Bağcılar, İstanbul, Turkey
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Parlati ALM, Nardi E, Marzano F, Madaudo C, Di Santo M, Cotticelli C, Agizza S, Abbellito GM, Perrone Filardi F, Del Giudice M, Annunziata FR, Martone I, Prastaro M, Paolillo S, Perrone Filardi P, Gargiulo P. Advancing Cardiovascular Diagnostics: The Expanding Role of CMR in Heart Failure and Cardiomyopathies. J Clin Med 2025; 14:865. [PMID: 39941536 PMCID: PMC11818251 DOI: 10.3390/jcm14030865] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/13/2025] [Revised: 01/25/2025] [Accepted: 01/26/2025] [Indexed: 02/16/2025] Open
Abstract
Cardiovascular magnetic resonance (CMR) imaging has become a cornerstone in the diagnosis, risk stratification, and management of cardiovascular disease (CVD), particularly heart failure (HF) and cardiomyopathies. Renowned as the gold standard for non-invasive quantification of ventricular volumes and ejection fraction, CMR delivers superior spatial and temporal resolution with excellent tissue-blood contrast. Recent advancements, including T1, T2, and T2* mapping, extracellular volume quantification, and late gadolinium enhancement, enable precise tissue characterization, allowing early detection of myocardial changes such as fibrosis, edema, and infiltration. These features provide critical insights into the pathophysiological mechanisms underlying HF phenotypes and diverse cardiomyopathies, enhancing diagnostic accuracy and guiding therapeutic decisions. This review explores the expanding role of CMR in CV disease, highlighting its diagnostic value in HF and in several cardiomyopathies, as well as its contribution to improving patient outcomes through detailed tissue characterization and prognosis.
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Affiliation(s)
| | - Ermanno Nardi
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Federica Marzano
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Cristina Madaudo
- Cardiology Unit, Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, University Hospital P. Giaccone, University of Palermo, 90127 Palermo, Italy
| | - Mariafrancesca Di Santo
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Ciro Cotticelli
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Simone Agizza
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Giuseppe Maria Abbellito
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Fabrizio Perrone Filardi
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Mario Del Giudice
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | | | - Isabel Martone
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Maria Prastaro
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Stefania Paolillo
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Pasquale Perrone Filardi
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Paola Gargiulo
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
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DI Gioia G, Ferrera A, Maestrini V, Monosilio S, Fiore R, Squeo MR, Pelliccia A. Revealing the unrevealed: echocardiography for non-ischemic scar tissue detection. J Sports Med Phys Fitness 2024; 64:1234-1238. [PMID: 39225027 DOI: 10.23736/s0022-4707.24.16267-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/04/2024]
Abstract
The detection of myocardial scar tissue profoundly influences athletes care and prognostic categorization. Athletes appear to be at risk of developing fatal arrhythmias when harboring a quiescent cardiac disorder. Early identification of disease in asymptomatic individuals through preparticipation screening is means to prevent these events. We presented a male marathon runner master athlete who came at our Department of Sports Medicine for a preparticipation screening. Baseline 12-lead standard electrocardiogram was normal. A maximal cycle ergometer exercise test revealed exercise-induced premature ventricular contractions (PVCs) with uncommon morphology. Echocardiography revealed an hyperechogenic zone at mid-basal posterior segments of the left ventricle. Twenty-four-hours ECG Holter monitoring, with training session, showed some isolated polymorphic PVCs even during training session. Cardiac magnetic resonance (CMR) confirmed the presence of a non-ischemic left ventricular scar (subepicardial) into the mid-basal segment of the posterior wall. Echocardiography is a first-line, economic, and accessible diagnostic test for athletes and it can be useful, when abnormalities are detected, to indicate further investigations, such as CMR. Although non-ischemic left ventricular scarring is difficult to detect on echocardiography, this event is sometimes possible and require further investigation when observed.
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Affiliation(s)
- Giuseppe DI Gioia
- National Italian Olympic Committee, Institute of Sports Medicine and Science, Rome, Italy -
- Department of Movement, Human and Health Sciences, University of Rome Foro Italico, Rome, Italy -
| | - Armando Ferrera
- Department of Clinical and Molecular Medicine, Sapienza University, Rome, Italy
| | - Viviana Maestrini
- National Italian Olympic Committee, Institute of Sports Medicine and Science, Rome, Italy
| | - Sara Monosilio
- National Italian Olympic Committee, Institute of Sports Medicine and Science, Rome, Italy
| | - Roberto Fiore
- National Italian Olympic Committee, Institute of Sports Medicine and Science, Rome, Italy
| | - Maria R Squeo
- National Italian Olympic Committee, Institute of Sports Medicine and Science, Rome, Italy
| | - Antonio Pelliccia
- National Italian Olympic Committee, Institute of Sports Medicine and Science, Rome, Italy
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Sokolska JM, Károlyi M, Hiestand DR, Gastl M, Weber L, Sokolski M, Kosmala W, Alkadhi H, Gruner C, Manka R. Myocardial Fibrosis Quantification Methods by Cardiovascular Magnetic Resonance Imaging in Patients with Fabry Disease. J Clin Med 2024; 13:5047. [PMID: 39274260 PMCID: PMC11395808 DOI: 10.3390/jcm13175047] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/14/2024] [Revised: 08/16/2024] [Accepted: 08/21/2024] [Indexed: 09/16/2024] Open
Abstract
Background/Objectives: The presence of late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) in patients with Fabry disease (FD) is a predictor of adverse cardiac events. The aim of this study was to establish the most reliable and reproducible technique for quantifying LGE in patients with FD. Methods: Twenty FD patients with LGE who underwent CMR on the same scanner and LGE sequence were included. LGE quantifications were done using gray-scale thresholds of 2, 3, 4, 5 and 6 standard deviations (SD) above the mean signal intensity of the remote myocardium, the full width at half maximum method (FWHM), visual assessment with threshold (VAT) and the fully manual method (MM). Results: The mean amount of fibrosis varied between quantification techniques from 36 ± 19 at 2SD to 2 ± 2 g using the FWHM (p < 0.0001). Intraobserver reliability was excellent for most methods, except for the FWHM which was good (ICC 0.84; all p < 0.05). Interobserver reliability was excellent for VAT (ICC 0.94) and good for other techniques (all p < 0.05). Intraobserver reproducibility showed the lowest coefficient of variation (CV, 6%) at 5SD and at 2SD and VAT (35% and 38%) for interobserver reproducibility. The FWHM revealed the highest CV (63% and 94%) for both intra- and interobserver reproducibility. Conclusions: The available methods for LGE quantification demonstrate good to excellent intra- and interobserver reproducibility in patients with FD. The most reliable and reproducible techniques were VAT and 5SD, whereas the FWHM was the least reliable in the setting of our study. The total amount of LGE varies strongly with the quantification technique used.
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Affiliation(s)
- Justyna M Sokolska
- Institute of Heart Diseases, Faculty of Medicine, Wroclaw Medical University, 50-556 Wroclaw, Poland
- University Heart Center, Department of Cardiology, University Hospital Zurich, 8091 Zurich, Switzerland
| | - Mihály Károlyi
- Diagnostic and Interventional Radiology, University Hospital Zurich, University of Zurich, 8091 Zurich, Switzerland
| | - Dana R Hiestand
- University Heart Center, Department of Cardiology, University Hospital Zurich, 8091 Zurich, Switzerland
| | - Mareike Gastl
- Department of Cardiology, Pulmonology and Vascular Medicine, Heinrich Heine University Düsseldorf, 40225 Düsseldorf, Germany
| | - Lucas Weber
- Diagnostic and Interventional Radiology, University Hospital Zurich, University of Zurich, 8091 Zurich, Switzerland
| | - Mateusz Sokolski
- Institute of Heart Diseases, Faculty of Medicine, Wroclaw Medical University, 50-556 Wroclaw, Poland
| | - Wojciech Kosmala
- Institute of Heart Diseases, Faculty of Medicine, Wroclaw Medical University, 50-556 Wroclaw, Poland
| | - Hatem Alkadhi
- Diagnostic and Interventional Radiology, University Hospital Zurich, University of Zurich, 8091 Zurich, Switzerland
| | - Christiane Gruner
- University Heart Center, Department of Cardiology, University Hospital Zurich, 8091 Zurich, Switzerland
| | - Robert Manka
- University Heart Center, Department of Cardiology, University Hospital Zurich, 8091 Zurich, Switzerland
- Diagnostic and Interventional Radiology, University Hospital Zurich, University of Zurich, 8091 Zurich, Switzerland
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Zhi Y, Gui FD, Xue M, Long YT, Miao W, Yi Y, Gao LC, Bing F, Pan SY. Focal ischemic myocardial fibrosis assessed by late gadolinium enhancement cardiovascular magnetic resonance in patients with hypertrophic cardiomyopathy. BMC Cardiovasc Disord 2024; 24:203. [PMID: 38594610 PMCID: PMC11003119 DOI: 10.1186/s12872-024-03859-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/11/2023] [Accepted: 03/25/2024] [Indexed: 04/11/2024] Open
Abstract
BACKGROUND In patients with hypertrophic cardiomyopathy (HCM), ischemic myocardial fibrosis assessed by late gadolinium enhancement (I-LGE) using cardiovascular magnetic resonance (CMR) have been reported. However, the clinical significance of I-LGE has not been completely understood. We aim to evaluate the I-LGE differ phenotypically from HCM without LGE or nonischemic myocardial fibrosis assessed by late gadolinium enhancement (NI-LGE) in the left ventricle (LV). METHODS The patients with HCM whom was underwent CMR were enrolled, using cine cardiac magnetic resonance to evaluate LV function and LGE to detect the myocardial fibrosis. Three groups were assorted: 1) HCM without LGE; 2) HCM with LGE involved the subendocardial layer was defined as I-LGE; 3) HCM with LGE not involved the subendocardial layer was defined as NI-LGE. RESULTS We enrolled 122 patients with HCM in the present study. LGE was detected in 58 of 122 (48%) patients with HCM, and 22 (18%) of patients reported I-LGE. HCM with I-LGE had increased higher left ventricular mass index (LVMI) (P < 0.0001) than HCM with NI-LGE or without LGE. In addition, HCM with I-LGE had a larger LV end- systolic volume (P = 0.045), lower LV ejection fraction (LVEF) (P = 0.026), higher LV myocardial mass (P < 0.001) and thicker LV wall (P < 0.001) more than HCM without LGE alone. The I-LGE were significantly associated with LVEF (OR: 0.961; P = 0.016), LV mass (OR: 1.028; P < 0.001), and maximal end-diastolic LVWT (OR: 1.567; P < 0.001). On multivariate analysis, LVEF (OR: 0.948; P = 0.013) and maximal end-diastolic LVWT (OR: 1.548; P = 0.001) were associated with higher risk for I-LGE compared to HCM without LGE. Noticeably, the maximal end-diastolic LVWT (OR: 1.316; P = 0.011) was the only associated with NI-LGE compared to HCM without LGE. CONCLUSIONS I-LGE is not uncommon in patients with HCM. HCM with I-LGE was associated with significant LV hypertrophy, extensive LGE and poor LV ejection fraction. We should consider focal ischemic myocardial fibrosis when applying LGE to risk stratification for HCM.
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Affiliation(s)
- Yang Zhi
- Department of Radiology, Chengdu Fifth People's Hospital, 33# Ma Shi Street, Chengdu, 611130, China
| | - Fu-Dan Gui
- Department of Cardiology, Chengdu Fifth People's Hospital, 33# Ma Shi Street, Chengdu, 611130, China
| | - Meng Xue
- Department of Radiology, Chengdu Fifth People's Hospital, 33# Ma Shi Street, Chengdu, 611130, China
| | - Yi-Tian Long
- Department of Radiology, Chengdu Fifth People's Hospital, 33# Ma Shi Street, Chengdu, 611130, China
| | - Wen Miao
- Department of Radiology, Chengdu Fifth People's Hospital, 33# Ma Shi Street, Chengdu, 611130, China
| | - You Yi
- Department of Radiology, Chengdu Fifth People's Hospital, 33# Ma Shi Street, Chengdu, 611130, China
| | - Liang-Chao Gao
- Department of Rheumatology and Immunology, Chengdu Fifth People's Hospital, Chengdu, China
| | - Fu Bing
- Department of Radiology, Chengdu Fifth People's Hospital, 33# Ma Shi Street, Chengdu, 611130, China.
| | - Shu-Yue Pan
- Department of Rheumatology and Immunology, Chengdu Fifth People's Hospital, Chengdu, China.
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Yang S, Zhao K, Yang K, Song J, Yu S, Wang J, Dong Z, Ma X, Yin G, Li J, Cheng H, Lu M, Chen X, Zhao S. Subendocardial Involvement as an Underrecognized LGE Subtype Related to Adverse Outcomes in Hypertrophic Cardiomyopathy. JACC Cardiovasc Imaging 2023; 16:1163-1177. [PMID: 37204388 DOI: 10.1016/j.jcmg.2023.03.011] [Citation(s) in RCA: 13] [Impact Index Per Article: 6.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/28/2022] [Revised: 02/16/2023] [Accepted: 03/15/2023] [Indexed: 05/20/2023]
Abstract
BACKGROUND Late gadolinium enhancement (LGE) has been established as an independent predictor for adverse outcomes in hypertrophic cardiomyopathy (HCM). However, the prevalence and clinical significance of some LGE subtypes have not been well demonstrated. OBJECTIVES In this study, the authors sought to investigate the prognostic value of subendocardium-involved LGE pattern and location of right ventricle insertion points (RVIPs) with LGE in HCM patients. METHODS In this single-center retrospective study, 497 consecutive HCM patients with LGE confirmed by cardiac magnetic resonance (CMR) were included. Subendocardium-involved LGE was defined as LGE involving subendocardium not corresponding to a coronary vascular distribution. Subjects with ischemic heart disease that would contribute to subendocardial LGE were excluded. Endpoints included a composite of heart failure-related events, arrhythmic events, and stroke. RESULTS Of the 497 patients, subendocardium-involved LGE and RVIP LGE were observed in 184 (37.0%) and 414 (83.3%), respectively. Extensive LGE (≥15% of left ventricular mass) was detected in 135 patients. During a median follow-up of 57.9 months, 66 patients (13.3%) experienced composite endpoints. Patients with extensive LGE had a significantly higher annual incidence of adverse events (5.1% vs 1.9% per year; P < 0.001). However, spline analysis showed that the association between LGE extent and HRs for adverse outcomes tended to be nonlinear. The risk of composite endpoint increased with percentage increase in LGE extent in patients with extensive LGE, whereas a similar trend was not observed in patients with nonextensive LGE (<15%). In patients with extensive LGE, LGE extent significantly correlated with composite endpoints (HR: 1.05; P = 0.03) after adjusting for left ventricular ejection fraction <50%, atrial fibrillation, and nonsustained ventricular tachycardia, whereas in patients with nonextensive LGE, subendocardium-involved LGE rather than LGE extent was independently associated with adverse outcomes (HR: 2.12; P = 0.03). RVIP LGE was not significantly associated with poor outcomes. CONCLUSIONS In HCM patients with nonextensive LGE, the presence of subendocardium-involved LGE rather than LGE extent is associated with unfavorable outcomes. Given that the prognostic value of extensive LGE has been broadly recognized, subendocardial involvement as an underrecognized LGE pattern shows the potential to improve risk stratification in HCM patients with nonextensive LGE.
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Affiliation(s)
- Shujuan Yang
- MR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, China
| | - Kankan Zhao
- Paul C. Lauterbur Research Center for Biomedical Imaging, Shenzhen Institutes of Advanced Technology, Chinese Academy of Sciences, Shenzhen, China
| | - Kai Yang
- MR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, China
| | - Jialin Song
- MR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, China
| | - Shiqin Yu
- MR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, China
| | - Jiaxin Wang
- MR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, China
| | - Zhixiang Dong
- MR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, China
| | - Xuan Ma
- MR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, China
| | - Gang Yin
- MR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, China
| | - Jinghui Li
- MR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, China
| | - Huaibing Cheng
- Department of Cardiology, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, China
| | - Minjie Lu
- MR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, China
| | - Xiuyu Chen
- MR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, China.
| | - Shihua Zhao
- MR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, China.
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Al Samarraie A, Petzl A, Cadrin-Tourigny J, Tadros R. Sudden Death Risk Assessment in Hypertrophic Cardiomyopathy Across the Lifespan: Reconciling the American and European Approaches. Card Electrophysiol Clin 2023; 15:367-378. [PMID: 37558306 DOI: 10.1016/j.ccep.2023.04.010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 08/11/2023]
Abstract
Hypertrophic cardiomyopathy (HCM) is the most prevalent inherited cardiac disease. Since the modern description of HCM more than seven decades ago, great focus has been placed on preventing its most catastrophic complication: sudden cardiac death (SCD). Implantable cardioverter-defibrillators (ICD) have been recognized to provide effective prophylactic therapy. Over the years, two leading societies, the European Society of Cardiology (ESC) and the American Heart Association/American College of Cardiology (AHA/ACC), have proposed risk stratification models to assess SCD in adults. European guidelines rely on a risk calculator, the HCM Risk-SCD, while American guidelines propose a stand-alone risk factor approach. Recently, risk prediction models were also developed in the pediatric population. This article reviews the latest recommendations on the risk stratification of SCD in HCM and summarises current indications for ICD use.
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Affiliation(s)
- Ahmad Al Samarraie
- Cardiovascular Genetics Centre, Montreal Heart Institute, 5000 Bélanger, Montreal, Quebec H1T 1C8, Canada; Faculty of Medicine, Université de Montréal, 2900 Edouard Montpetit, Montreal, Quebec H3T 1J4, Canada
| | - Adrian Petzl
- Cardiovascular Genetics Centre, Montreal Heart Institute, 5000 Bélanger, Montreal, Quebec H1T 1C8, Canada; Faculty of Medicine, Université de Montréal, 2900 Edouard Montpetit, Montreal, Quebec H3T 1J4, Canada
| | - Julia Cadrin-Tourigny
- Cardiovascular Genetics Centre, Montreal Heart Institute, 5000 Bélanger, Montreal, Quebec H1T 1C8, Canada; Faculty of Medicine, Université de Montréal, 2900 Edouard Montpetit, Montreal, Quebec H3T 1J4, Canada
| | - Rafik Tadros
- Cardiovascular Genetics Centre, Montreal Heart Institute, 5000 Bélanger, Montreal, Quebec H1T 1C8, Canada; Faculty of Medicine, Université de Montréal, 2900 Edouard Montpetit, Montreal, Quebec H3T 1J4, Canada.
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Gu ZY, Qian YF, Chen BH, Wu CW, Zhao L, Xue S, Zhao L, Wu LM, Wang YY. Late gadolinium enhancement entropy as a new measure of myocardial tissue heterogeneity for prediction of adverse cardiac events in patients with hypertrophic cardiomyopathy. Insights Imaging 2023; 14:138. [PMID: 37603140 PMCID: PMC10441833 DOI: 10.1186/s13244-023-01479-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/30/2023] [Accepted: 07/04/2023] [Indexed: 08/22/2023] Open
Abstract
OBJECTIVES Entropy is a new late gadolinium enhanced (LGE) cardiac magnetic resonance (CMR)-derived parameter that is independent of signal intensity thresholds. Entropy can be used to measure myocardial tissue heterogeneity by comparing full pixel points of tissue images. This study investigated the incremental prognostic value of left ventricular (LV) entropy in patients with hypertrophic cardiomyopathy (HCM). METHODS This study enrolled 337 participants with HCM who underwent 3.0-T CMR. The LV entropy was obtained by calculating the probability distribution of the LV myocardial pixel signal intensities of the LGE sequence. Patients who underwent CMR imaging were followed up for endpoints. The primary endpoint was defined as readmission to the hospital owing to heart failure. The secondary endpoint was the composite of the primary endpoint, sudden cardiac death and non-cardiovascular death. RESULTS During the median follow-up of 24 months ± 13 (standard deviation), 43 patients who reached the primary and secondary endpoints had a higher entropy (6.20 ± 0.45, p < 0.001). The patients with increased entropy (≥ 5.587) had a higher risk of the primary and secondary endpoints, compared with HCM patients with low entropy (p < 0.001 for both). In addition, Cox analysis showed that LV entropy provided significant prognostic value for predicting both primary and secondary endpoints (HR: 1.291 and 1.273, all p < 0.001). Addition of LV entropy to the multivariable model improved model performance and risk reclassification (p < 0.05). CONCLUSION LV entropy assessed by CMR was an independent predictor of primary and secondary endpoints. LV entropy assessment contributes to improved risk stratification in patients with HCM. CRITICAL RELEVANCE STATEMENT Myocardial heterogeneity reflected by entropy the derived parameter of LGE has prognostic value for adverse events in HCM. The measurement of LV entropy helped to identify patients with HCM who were at risk for heart failure and sudden cardiac death. KEY POINTS • Left ventricular entropy can reflect myocardial heterogeneity in HCM patients. • Left ventricular entropy was significantly higher in HCM patients who reached endpoint events. • Left ventricular entropy helps to predict the occurrence of heart failure and death in HCM patients.
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Affiliation(s)
- Zi-Yi Gu
- Department of Cardiovascular Surgery, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China
| | - Yu-Fan Qian
- Department of Radiology, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China
| | - Bing-Hua Chen
- Department of Radiology, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China
| | - Chong-Wen Wu
- Department of Radiology, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China
| | - Lei Zhao
- Department of Cardiovascular Surgery, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China
| | - Song Xue
- Department of Cardiovascular Surgery, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China
| | - Lei Zhao
- Department of Radiology, Beijing Anzhen Hospital, Capital Medical University, Beijing, 100029, China.
| | - Lian-Ming Wu
- Department of Radiology, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China.
| | - Yong-Yi Wang
- Department of Cardiovascular Surgery, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China.
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She J, Zhao S, Chen Y, Zeng M, Jin H. Detecting Regional Fibrosis in Hypertrophic Cardiomyopathy: The Utility of Myocardial Strain Based on Cardiac Magnetic Resonance. Acad Radiol 2023; 30:230-238. [PMID: 35469720 DOI: 10.1016/j.acra.2022.03.022] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/04/2022] [Revised: 03/23/2022] [Accepted: 03/24/2022] [Indexed: 01/11/2023]
Abstract
RATIONALE AND OBJECTIVES The value of myocardial strain for reflecting fibrosis in patients with hypertrophic cardiomyopathy (HCM) on cardiac magnetic resonance (CMR) has not been definite. We aim to explore whether there are underlying non-contrast parameters to evaluate myocardial fibrosis and screen which may be the best. MATERIALS AND METHODS We retrospectively included 127 HCM patients (89 men; average age 46.6 ± 15.6 years) and 30 healthy controls (20 men; average age 52.0 ± 13.2 years) who have undergone late gadolinium enhancement (LGE) CMR. Next, 127 HCM patients were divided randomly into two sets including training cohort and validation cohort. Strain and imaging parameters were measured and analyzed statistically. RESULTS Based on univariate and multivariate analysis, segmental circumferential strain (SCS) (p < 0.001) and maximal wall thickness (MWT) (p < 0.001) may differentiate myocardial segments with or without LGE as significant biomarkers for both sets. The area under the curve (AUC) was 0.803 (95% CI 0.785-0.820) for SCS and 0.777 (95% CI 0.759-0.795) for MWT to identify myocardial fibrosis. When combining SCS >-13.9% and MWT >16.4mm, the specificity of the model (AUC = 0.779; 95% CI 0.760-0.796) achieved the highest 93.9%, with a sensitivity of 61.8%. CONCLUSION Strain analysis in HCM holds promise for myocardial fibrosis detection and SCS is the best strain parameter based on CMR. Nevertheless, the model of combining SCS and MWT could achieve the highest specificity for fibrotic diagnosis.
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Affiliation(s)
- Jiaqi She
- Department of Radiology, Zhongshan Hospital, Fudan University and Shanghai Institute of Medical Imaging, Shanghai, China; Department of Medical Imaging, Shanghai Medical school, Fudan University, Shanghai, China
| | - Shihai Zhao
- Department of Radiology, Zhongshan Hospital, Fudan University and Shanghai Institute of Medical Imaging, Shanghai, China; Department of Medical Imaging, Shanghai Medical school, Fudan University, Shanghai, China
| | - Yinyin Chen
- Department of Radiology, Zhongshan Hospital, Fudan University and Shanghai Institute of Medical Imaging, Shanghai, China; Department of Medical Imaging, Shanghai Medical school, Fudan University, Shanghai, China
| | - Mengsu Zeng
- Department of Radiology, Zhongshan Hospital, Fudan University and Shanghai Institute of Medical Imaging, Shanghai, China; Department of Medical Imaging, Shanghai Medical school, Fudan University, Shanghai, China
| | - Hang Jin
- Department of Radiology, Zhongshan Hospital, Fudan University and Shanghai Institute of Medical Imaging, Shanghai, China; Department of Medical Imaging, Shanghai Medical school, Fudan University, Shanghai, China.
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10
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Multimodality Imaging in Sarcomeric Hypertrophic Cardiomyopathy: Get It Right…on Time. LIFE (BASEL, SWITZERLAND) 2023; 13:life13010171. [PMID: 36676118 PMCID: PMC9863627 DOI: 10.3390/life13010171] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 11/16/2022] [Revised: 12/25/2022] [Accepted: 12/29/2022] [Indexed: 01/09/2023]
Abstract
Hypertrophic cardiomyopathy (HCM) follows highly variable paradigms and disease-specific patterns of progression towards heart failure, arrhythmias and sudden cardiac death. Therefore, a generalized standard approach, shared with other cardiomyopathies, can be misleading in this setting. A multimodality imaging approach facilitates differential diagnosis of phenocopies and improves clinical and therapeutic management of the disease. However, only a profound knowledge of the progression patterns, including clinical features and imaging data, enables an appropriate use of all these resources in clinical practice. Combinations of various imaging tools and novel techniques of artificial intelligence have a potentially relevant role in diagnosis, clinical management and definition of prognosis. Nonetheless, several barriers persist such as unclear appropriate timing of imaging or universal standardization of measures and normal reference limits. This review provides an overview of the current knowledge on multimodality imaging and potentialities of novel tools, including artificial intelligence, in the management of patients with sarcomeric HCM, highlighting the importance of specific "red alerts" to understand the phenotype-genotype linkage.
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11
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Pham JH, Giudicessi JR, Tweet MS, Boucher L, Newman DB, Geske JB. Tale of two hearts: a TNNT2 hypertrophic cardiomyopathy case report. Front Cardiovasc Med 2023; 10:1167256. [PMID: 37180798 PMCID: PMC10174446 DOI: 10.3389/fcvm.2023.1167256] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/16/2023] [Accepted: 04/07/2023] [Indexed: 05/16/2023] Open
Abstract
Hypertrophic cardiomyopathy (HCM) is a heritable cardiomyopathy that is predominantly caused by pathogenic mutations in sarcomeric proteins. Here we report two individuals, a mother and her daughter, both heterozygous carriers of the same HCM-causing mutation in cardiac Troponin T (TNNT2). Despite sharing an identical pathogenic variant, the two individuals had very different manifestations of the disease. While one patient presented with sudden cardiac death, recurrent tachyarrhythmia, and findings of massive left ventricular hypertrophy, the other patient manifested with extensive abnormal myocardial delayed enhancement despite normal ventricular wall thickness and has remained relatively asymptomatic. Recognition of the marked incomplete penetrance and variable expressivity possible in a single TNNT2-positive family has potential to guide HCM patient care.
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Affiliation(s)
- Justin H. Pham
- Mayo Clinic Alix School of Medicine, Mayo Clinic, Rochester, MN, United States
| | - John R. Giudicessi
- Department of Cardiovascular Medicine, Mayo Clinic, Rochester MN, United States
- Department of Molecular Pharmacology and Experimental Therapeutics, Mayo Clinic, Rochester, MN, United States
| | - Marysia S. Tweet
- Department of Cardiovascular Medicine, Mayo Clinic, Rochester MN, United States
| | - Lauren Boucher
- Department of Cardiovascular Medicine, Mayo Clinic, Rochester MN, United States
| | - D. Brian Newman
- Department of Cardiovascular Medicine, Mayo Clinic, Rochester MN, United States
| | - Jeffrey B. Geske
- Department of Cardiovascular Medicine, Mayo Clinic, Rochester MN, United States
- Correspondence: Jeffrey B. Geske
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12
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Cardiac Hypertrophy and Related Dysfunctions in Cushing Syndrome Patients-Literature Review. J Clin Med 2022; 11:jcm11237035. [PMID: 36498610 PMCID: PMC9739690 DOI: 10.3390/jcm11237035] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/01/2022] [Revised: 11/19/2022] [Accepted: 11/21/2022] [Indexed: 11/29/2022] Open
Abstract
The survival rate of adrenal Cushing syndrome patients has been greatly increased because of the availability of appropriate surgical and pharmacological treatments. Nevertheless, increased possibility of a heart attack induced by a cardiovascular event remains a major risk factor for the survival of affected patients. In experimental studies, hypercortisolemia has been found to cause cardiomyocyte hypertrophy via glucocorticoid receptor activation, including the possibility of cross talk among several hypertrophy signals related to cardiomyocytes and tissue-dependent regulation of 11β-hydroxysteroid dehydrogenase type 1. However, the factors are more complex in clinical cases, as both geometric and functional impairments leading to heart failure have been revealed, and their associations with a wide range of factors such as hypertension are crucial. In addition, knowledge regarding such alterations in autonomous cortisol secretion, which has a high risk of leading to heart attack as well as overt Cushing syndrome, is quite limited. When considering the effects of treatment, partial improvement of structural alterations is expected, while functional disorders are controversial. Therefore, whether the normalization of excess cortisol attenuates the risk related to cardiac hypertrophy has yet to be fully elucidated.
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13
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Doste R, Coppini R, Bueno-Orovio A. Remodelling of potassium currents underlies arrhythmic action potential prolongation under beta-adrenergic stimulation in hypertrophic cardiomyopathy. J Mol Cell Cardiol 2022; 172:120-131. [PMID: 36058298 DOI: 10.1016/j.yjmcc.2022.08.361] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/28/2022] [Revised: 08/15/2022] [Accepted: 08/27/2022] [Indexed: 12/14/2022]
Abstract
Hypertrophic cardiomyopathy (HCM) patients often present an enhanced arrhythmogenicity that can lead to lethal arrhythmias, especially during exercise. Recent studies have indicated an abnormal response of HCM cardiomyocytes to β-adrenergic receptor stimulation (β-ARS), with prolongation of their action potential rather than shortening. The mechanisms underlying this aberrant response to sympathetic stimulation and its possible proarrhythmic role remain unknown. The aims of this study are to investigate the key ionic mechanisms underlying the HCM abnormal response to β-ARS and the resultant repolarisation abnormalities using human-based experimental and computational methodologies. We integrated and calibrated the latest models of human ventricular electrophysiology and β-ARS using experimental measurements of human adult cardiomyocytes from control and HCM patients. Our major findings include: (1) the developed in silico models of β-ARS capture the behaviour observed in the experimental data, including the aberrant response of HCM cardiomyocytes to β-ARS; (2) the reduced increase of potassium currents under β-ARS was identified as the main mechanism of action potential prolongation in HCM, rather than a more sustained inward calcium current; (3) action potential duration differences between healthy and HCM cardiomyocytes were increased upon β-ARS, while endocardial to epicardial differences in HCM cardiomyocytes were reduced; (4) models presenting repolarisation abnormalities were characterised by downregulation of the rapid delayed rectifier potassium current and the sodium‑potassium pump, while inward currents were upregulated. In conclusion, our results identify causal relationships between the HCM phenotype and its arrhythmogenic response to β-ARS through the downregulation of potassium currents.
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Affiliation(s)
- Ruben Doste
- Department of Computer Science, BHF Centre of Research Excellence, University of Oxford, Oxford, United Kingdom
| | | | - Alfonso Bueno-Orovio
- Department of Computer Science, BHF Centre of Research Excellence, University of Oxford, Oxford, United Kingdom.
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14
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Ranjan P, Ro R, Lerakis S. Multislice Computed Tomography (MSCT) and Cardiovascular Magnetic Resonance (CMR) Imaging for Coronary and Structural Heart Disease. Interv Cardiol 2022. [DOI: 10.1002/9781119697367.ch10] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022] Open
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15
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Sivalokanathan S. The Role of Cardiovascular Magnetic Resonance Imaging in the Evaluation of Hypertrophic Cardiomyopathy. Diagnostics (Basel) 2022; 12:diagnostics12020314. [PMID: 35204405 PMCID: PMC8871211 DOI: 10.3390/diagnostics12020314] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/07/2021] [Revised: 01/08/2022] [Accepted: 01/25/2022] [Indexed: 01/19/2023] Open
Abstract
Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disorder, affecting 1 out of 500 adults globally. It is a widely heterogeneous disorder characterized by a range of phenotypic expressions, and is most often identified by non-invasive imaging that includes echocardiography and cardiovascular magnetic resonance imaging (CMR). Within the last two decades, cardiac magnetic resonance imaging (MRI) has emerged as the defining tool for the characterization and prognostication of cardiomyopathies. With a higher image quality, spatial resolution, and the identification of morphological variants of HCM, CMR has become the gold standard imaging modality in the assessment of HCM. Moreover, it has been crucial in its management, as well as adding prognostic information that clinical history nor other imaging modalities may not provide. This literature review addresses the role and current applications of CMR, its capacity in evaluating HCM, and its limitations.
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Affiliation(s)
- Sanjay Sivalokanathan
- Internal Medicine, Pennsylvania Hospital, University of Pennsylvania Health System, Philadelphia, PA 19107, USA;
- Cardiovascular Clinical Academic Group, St. George’s University of London and St George’s University Hospitals NHS Foundation Trust, London SW17 0RE, UK
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16
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Cardiovascular Magnetic Resonance (CMR) for the Evaluation of Myocardial Infarction in Patients with Non-obstructive Coronary Artery Disease (MINOCA). CURRENT RADIOLOGY REPORTS 2021. [DOI: 10.1007/s40134-021-00384-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/20/2022]
Abstract
Abstract
Purpose of Review
Myocardial infarction in the absence of coronary artery disease is caused by a variety of clinical conditions, so it is important to detect the specific causes in order to perform a better prognostic stratification of these patients. The aim of this review is to summarize the most important findings that established the role of CMR (cardiovascular magnetic resonance) to detect the MINOCA (myocardial infarction with non-obstructive arteries) patients and the importance to differentiate them from myocardial infarction patients.
Recent Findings
The role of CMR is crucial to diagnose the principal diseases involved in MINOCA, as demonstrated. The several MR sequences used in all the MINOCA patients showed different results for all the different causes of MINOCA and, surely, high-resolution MR with gadolinium enhancement has been considered the best method to differentiate the transmural lesions.
Summary
Another fundamental aspect to be considered is the experience of CMR radiologists, which represent the most important element for the right diagnosis of MINOCA. Surely, in the future, CMR will be the most important technique of choice for MINOCA patients, playing a key role in their management.
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17
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Mancio J, Pashakhanloo F, El-Rewaidy H, Jang J, Joshi G, Csecs I, Ngo L, Rowin E, Manning W, Maron M, Nezafat R. Machine learning phenotyping of scarred myocardium from cine in hypertrophic cardiomyopathy. Eur Heart J Cardiovasc Imaging 2021; 23:532-542. [PMID: 33779725 DOI: 10.1093/ehjci/jeab056] [Citation(s) in RCA: 21] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/07/2020] [Indexed: 12/12/2022] Open
Abstract
AIMS Cardiovascular magnetic resonance (CMR) with late-gadolinium enhancement (LGE) is increasingly being used in hypertrophic cardiomyopathy (HCM) for diagnosis, risk stratification, and monitoring. However, recent data demonstrating brain gadolinium deposits have raised safety concerns. We developed and validated a machine-learning (ML) method that incorporates features extracted from cine to identify HCM patients without fibrosis in whom gadolinium can be avoided. METHODS AND RESULTS An XGBoost ML model was developed using regional wall thickness and thickening, and radiomic features of myocardial signal intensity, texture, size, and shape from cine. A CMR dataset containing 1099 HCM patients collected using 1.5T CMR scanners from different vendors and centres was used for model development (n=882) and validation (n=217). Among the 2613 radiomic features, we identified 7 features that provided best discrimination between +LGE and -LGE using 10-fold stratified cross-validation in the development cohort. Subsequently, an XGBoost model was developed using these radiomic features, regional wall thickness and thickening. In the independent validation cohort, the ML model yielded an area under the curve of 0.83 (95% CI: 0.77-0.89), sensitivity of 91%, specificity of 62%, F1-score of 77%, true negatives rate (TNR) of 34%, and negative predictive value (NPV) of 89%. Optimization for sensitivity provided sensitivity of 96%, F2-score of 83%, TNR of 19% and NPV of 91%; false negatives halved from 4% to 2%. CONCLUSION An ML model incorporating novel radiomic markers of myocardium from cine can rule-out myocardial fibrosis in one-third of HCM patients referred for CMR reducing unnecessary gadolinium administration.
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Affiliation(s)
- Jennifer Mancio
- Department of Medicine, Beth Israel Deaconess Medical Centre and Harvard Medical School, 330 Brookline Avenue, Boston, MA 02215, USA
| | - Farhad Pashakhanloo
- Department of Medicine, Beth Israel Deaconess Medical Centre and Harvard Medical School, 330 Brookline Avenue, Boston, MA 02215, USA
| | - Hossam El-Rewaidy
- Department of Medicine, Beth Israel Deaconess Medical Centre and Harvard Medical School, 330 Brookline Avenue, Boston, MA 02215, USA.,Department of Computer Science, Technical University of Munich, Arcisstraße 21, 80333 Munich, Germany
| | - Jihye Jang
- Department of Medicine, Beth Israel Deaconess Medical Centre and Harvard Medical School, 330 Brookline Avenue, Boston, MA 02215, USA.,Department of Computer Science, Technical University of Munich, Arcisstraße 21, 80333 Munich, Germany
| | - Gargi Joshi
- Department of Medicine, Beth Israel Deaconess Medical Centre and Harvard Medical School, 330 Brookline Avenue, Boston, MA 02215, USA
| | - Ibolya Csecs
- Department of Medicine, Beth Israel Deaconess Medical Centre and Harvard Medical School, 330 Brookline Avenue, Boston, MA 02215, USA
| | - Long Ngo
- Department of Medicine, Beth Israel Deaconess Medical Centre and Harvard Medical School, 330 Brookline Avenue, Boston, MA 02215, USA.,Department of Biostatistics, Harvard T.H. Chan School of Public Health, 677 Huntington Ave, Boston, MA 02115, USA
| | - Ethan Rowin
- HCM Institute, Division of Cardiology, Tufts Medical Centre, 860 Washington St Building, 6th Floor, Boston, MA 02111, USA
| | - Warren Manning
- Department of Medicine, Beth Israel Deaconess Medical Centre and Harvard Medical School, 330 Brookline Avenue, Boston, MA 02215, USA.,Department of Radiology, Beth Israel Deaconess Medical Centre and Harvard Medical School, 330 Brookline Avenue, Boston, MA 02215, USA
| | - Martin Maron
- HCM Institute, Division of Cardiology, Tufts Medical Centre, 860 Washington St Building, 6th Floor, Boston, MA 02111, USA
| | - Reza Nezafat
- Department of Medicine, Beth Israel Deaconess Medical Centre and Harvard Medical School, 330 Brookline Avenue, Boston, MA 02215, USA
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18
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Österberg AW, Östman-Smith I, Jablonowski R, Carlsson M, Green H, Gunnarsson C, Liuba P, Fernlund E. High ECG Risk-Scores Predict Late Gadolinium Enhancement on Magnetic Resonance Imaging in HCM in the Young. Pediatr Cardiol 2021; 42:492-500. [PMID: 33515326 DOI: 10.1007/s00246-020-02506-9] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/30/2020] [Accepted: 11/17/2020] [Indexed: 01/14/2023]
Abstract
An ECG risk-score has been described that predicts high risk of subsequent cardiac arrest in young patients with hypertrophic cardiomyopathy (HCM). Myocardial fibrosis measured by cardiac magnetic resonance (CMR) late gadolinium enhancement (LGE) also affects prognosis. We assessed whether an ECG risk-score could be used as an indicator of myocardial fibrosis or perfusion deficit on CMR in HCM. In total 42 individuals (7-31 years); 26 HCM patients, seven genotype-positive, phenotype-negative individuals at risk of HCM (first-degree relatives) and nine healthy volunteers, underwent CMR to identify, and grade extent of, myocardial fibrosis and perfusion defect. 12-lead ECG was used for calculating the ECG risk-score (grading 0-14p). High-risk ECG (risk-score > 5p) occurred only in the HCM group (9/26), and the proportion was significantly higher vs mutation carriers combined with healthy volunteers (0/16, p = 0.008). Extent of LGE correlated to the ECG-score (R2 = 0.47, p = 0.001) in sarcomeric mutations. In low-risk ECG-score patients (0-2p), median percent of myocardium showing LGE (LGE%LVM) were: 0% [interquartile range, IQR, 0-0%], in intermediate-risk (3-5p): 5.4% [IQR 0-13.5%] and in high-risk (6-14p): 10.9% [IQR 4.2-12.3%]. ECG-score > 2p had a sensitivity and specificity of 79% and 84% to detect positive LGE on CMR and 77% vs. 75% to detect perfusion defects in sarcomeric mutations carriers. In patients with myocardial fibrosis as identified by LGE, median ECG risk-score was 8p [range 3-10p]. In conclusions, ECG risk-score > 2 p could be used as a cut-off for screening of myocardial fibrosis. Thus ECG risk-score is an inexpensive complementary tool in risk stratification of HCM in the young.
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Affiliation(s)
- Anna Wålinder Österberg
- Crown Princess Victoria Children's Hospital, Department of Biomedical and Clinical Sciences, Department of Pediatrics, Linköping University, 581 85, Linköping, Sweden
| | - Ingegerd Östman-Smith
- Department of Pediatrics, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden
| | - Robert Jablonowski
- Clinical Physiology, Department of Clinical Sciences, Lund University, Lund, Sweden
| | - Marcus Carlsson
- Clinical Physiology, Department of Clinical Sciences, Lund University, Lund, Sweden
| | - Henrik Green
- Department of Forensic Genetics and Forensic Toxicology, National Board of Forensic Medicine, Linköping, Sweden.,Division of Drug Research, Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden
| | - Cecilia Gunnarsson
- Department of Clinical Genetics, Department of Biomedical and Clinical Sciences, Centre for Rare Diseases in South East Region of Sweden, Linköping University, Linköping, Sweden
| | - Petru Liuba
- Pediatric Heart Centre, Skåne University Hospital and Department of Clinical Sciences, Lund University, Lund, Sweden
| | - Eva Fernlund
- Crown Princess Victoria Children's Hospital, Department of Biomedical and Clinical Sciences, Department of Pediatrics, Linköping University, 581 85, Linköping, Sweden. .,Pediatric Heart Centre, Skåne University Hospital and Department of Clinical Sciences, Lund University, Lund, Sweden.
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19
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The Clinical Prognosis of Presence and Location of Late Gadolinium Enhancement by Cardiac Magnetic Resonance Imaging in Patients with Hypertrophic Cardiomyopathy: a Single-Center Cohort Study. J Cardiovasc Transl Res 2021; 14:1001-1016. [PMID: 33629154 DOI: 10.1007/s12265-021-10107-x] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/10/2020] [Accepted: 02/15/2021] [Indexed: 01/07/2023]
Abstract
Increasing data have indicated that late gadolinium enhancement (LGE) in cardiac magnetic resonance (CMR) images is associated with the clinical prognosis of hypertrophic cardiomyopathy (HCM). Recently, pioneer studies indicated that the location of LGE in CMR images also had potential predictive value for HCM prognosis. The aim of the present study was to investigate the prognostic value of the location of LGE for HCM. This present cohort study included 557 HCM patients who underwent LGE-CMR imaging, and the LGE location was classified as LGE in interventricular septum only (IVS-LGE) and LGE outside the IVS with or without IVS involvement (other than IVS-LGE). All-cause mortality, cardiovascular mortality/cardiac transplantation, and sudden cardiac death (SCD) were evaluated. During a mean follow-up time of 83.0±37.8 months, there was a significantly higher all-cause mortality, cardiovascular mortality/cardiac transplantation, and SCD in patients with other than IVS-LGE than in those with IVS-LGE. Multivariate Cox regression suggested that other than IVS-LGE were one of independent prognostic predictors. Risk reclassification for prognosis showed that there were no differences between the prediction values of the presence of LGE and the location of LGE. The presence and location of LGE in CMR images are equally independent prognostic predictors of HCM, and other than IVS-LGE location is associated with an adverse clinical prognosis. Prognosis Trial Registration: ChiCTR-ONRC-11001902.
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20
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Polaková E, Liebregts M, Marková N, Adla T, Kara B, Ten Berg J, Bonaventura J, Veselka J. Effectiveness of alcohol septal ablation for hypertrophic obstructive cardiomyopathy in patients with late gadolinium enhancement on cardiac magnetic resonance. Int J Cardiol 2020; 319:101-105. [PMID: 32682963 DOI: 10.1016/j.ijcard.2020.06.049] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/20/2020] [Revised: 05/21/2020] [Accepted: 06/24/2020] [Indexed: 01/20/2023]
Abstract
BACKGROUND According to European guidelines, alcohol septal ablation (ASA) for hypertrophic obstructive cardiomyopathy (HOCM) may be less effective in patients with extensive septal scarring on cardiac magnetic resonance (CMR). This study aimed to analyze the impact of late gadolinium enhancement (LGE) on CMR on the effectiveness of ASA. METHOD We conducted an observational retrospective study involving adult patients with symptomatic drug-refractory HOCM who underwent CMR before ASA at two European centres from May 2010 through June 2019. Patients were compared in binary format based on LGE presence. Moreover, a subanalysis focused on patients with septal fibrosis was performed. The effectiveness of ASA was evaluated by echocardiographic, ECG and clinical findings. RESULTS Of the 113 study patients, 54 (48%) had LGE on CMR. The LGE quantification performed in 29 patients revealed septal fibrosis in 17. The mean follow-up was 4.4 ± 2.6 years. Baseline parameters were similar between groups except for basal septal thickness that was greater in LGE+ group (21.1 ± 3.9 mm for LGE+ vs. 19.2 ± 3.2 mm for LGE-: p = .005). ASA improved symptoms in all groups and reduced left ventricular outflow tract obstruction (LVOTO) (delta gradient reduction: LGE+: 62 ± 37.3%; septal LGE+: 75.6 ± 20.8%; LGE-: 72.5 ± 21.0%). However, 13% of the LGE+ and 2% of the LGE- group had residual LVOTO above 30 mmHg (p = .027). CONCLUSION ASA was effective in all patients with HOCM, whether they had LGE on CMR or not and whether they had septal fibrosis or not.
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Affiliation(s)
- Eva Polaková
- Department of Cardiology, Second Medical School, Charles University, Motol University Hospital, Prague, Czech Republic.
| | - Max Liebregts
- Department of Cardiology, St.Antonius Hospital, Nieuwegein, the Netherlands
| | - Natália Marková
- Department of Radiology, Second Medical School, Charles University, Motol University Hospital, Prague, Czech Republic
| | - Theodor Adla
- Department of Radiology, Second Medical School, Charles University, Motol University Hospital, Prague, Czech Republic
| | - Basak Kara
- Department of Cardiology, St.Antonius Hospital, Nieuwegein, the Netherlands
| | - Jurriën Ten Berg
- Department of Cardiology, St.Antonius Hospital, Nieuwegein, the Netherlands
| | - Jiří Bonaventura
- Department of Cardiology, Second Medical School, Charles University, Motol University Hospital, Prague, Czech Republic
| | - Josef Veselka
- Department of Cardiology, Second Medical School, Charles University, Motol University Hospital, Prague, Czech Republic
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21
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Kamp NJ, Chery G, Kosinski AS, Desai MY, Wazni O, Schmidler GS, Patel M, Lopes RD, Morin DP, Al-Khatib SM. Risk stratification using late gadolinium enhancement on cardiac magnetic resonance imaging in patients with hypertrophic cardiomyopathy: A systematic review and meta-analysis. Prog Cardiovasc Dis 2020; 66:10-16. [PMID: 33171204 DOI: 10.1016/j.pcad.2020.11.001] [Citation(s) in RCA: 23] [Impact Index Per Article: 4.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/01/2020] [Accepted: 11/01/2020] [Indexed: 11/19/2022]
Abstract
Background The role of late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (c-MRI) for predicting outcomes of patients with hypertrophic cardiomyopathy (HCM) has been debated. Methods We searched PubMed and Embase and various published bibliographies for prospective studies published in English between January 1990 and February 2019. Two investigators screened 2646 abstracts and full-text articles for inclusion and relevant outcomes. We then performed a systematic review and meta-analysis to calculate pooled odds ratios for LGE on c-MRI and a pooled sensitivity and specificity analysis. Results Our systematic review included 8 prospective studies and 3808 patients. LGE positivity was associated with higher odds of the endpoint of sudden cardiac death (SCD;OR 1.69, 95%CI 1.03-2.78), aborted SCD or appropriate implantable cardioverter- defibrillator (ICD) discharge (OR 3.27 [1.75-6.10]), SCD or aborted SCD or appropriate ICD discharge (OR 2.32 [1.56-3.43]), and all-cause mortality (OR 2.10 [CI 1.00-4.41]). The pooled sensitivity and specificity of positive LGE on c-MRI for SCD were 65% and 42%, respectively; for aborted SCD or appropriate ICD discharge, 79% and 39%; for SCD or aborted SCD or appropriate ICD discharge, 74% and 39%; and for all-cause mortality, 78% and 39%. Conclusion In patients with HCM, LGE on c-MRI is a strong predictor of arrhythmic outcomes including SCD, aborted SCD, and appropriate ICD therapy. These data support the routine use of LGE on c-MRI as a marker of SCD risk in this population.
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MESH Headings
- Adult
- Arrhythmias, Cardiac/etiology
- Arrhythmias, Cardiac/mortality
- Arrhythmias, Cardiac/prevention & control
- Cardiomyopathy, Hypertrophic/complications
- Cardiomyopathy, Hypertrophic/diagnostic imaging
- Cardiomyopathy, Hypertrophic/mortality
- Cardiomyopathy, Hypertrophic/therapy
- Contrast Media
- Death, Sudden, Cardiac/etiology
- Death, Sudden, Cardiac/prevention & control
- Female
- Gadolinium
- Humans
- Magnetic Resonance Imaging
- Male
- Middle Aged
- Predictive Value of Tests
- Prognosis
- Risk Assessment
- Risk Factors
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Affiliation(s)
| | | | - Andrzej S Kosinski
- Department of Biostatistics & Bioinformatics, Duke University, Durham, NC, USA
| | | | | | | | - Manesh Patel
- Duke Clinical Research Institute, Durham, NC, USA
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22
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Begg A, Dahiya G, Kyvernitakis A, Biederman RWW. The use of contrast‐enhanced transthoracic echocardiography for spiral‐variant hypertrophic cardiomyopathy. Echocardiography 2020; 37:1873-1876. [DOI: 10.1111/echo.14871] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/01/2020] [Revised: 09/08/2020] [Accepted: 09/09/2020] [Indexed: 11/29/2022] Open
Affiliation(s)
- Andrew Begg
- Internal Medicine Allegheny General Hospital Pittsburgh PA USA
| | - Garima Dahiya
- Internal Medicine Allegheny General Hospital Pittsburgh PA USA
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23
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Abstract
Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiac condition and highly heterogeneous. Echocardiography and genetic and clinical screening have led to detection in women of childbearing age. Maternal and fetal outcomes among women with HCM are favorable. Genetic counseling is recommended. Prepregnancy clinical evaluation and risk assessment are paramount in ensuring optimal outcomes. Most women carry moderate risk of morbidity, have clinical evaluations and echocardiography each trimester, and deliver vaginally. Those who are symptomatic or have significant left ventricular outflow obstruction or recurrent arrhythmias prior to pregnancy are at higher risk and should be monitored at least monthly.
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Affiliation(s)
- Sara Saberi
- Department of Internal Medicine, Division of Cardiovascular Medicine, University of Michigan School of Medicine, 1500 East Medical Center Drive, CVC Suite 2364, Ann Arbor, MI 48109-5853, USA.
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24
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Kubo T, Ochi Y, Baba Y, Sugiura K, Takahashi A, Hirota T, Yamanaka S, Yamasaki N, Doi YL, Kitaoka H. Elevation of high-sensitivity cardiac troponin T and left ventricular remodelling in hypertrophic cardiomyopathy. ESC Heart Fail 2020; 7:3593-3600. [PMID: 33047518 PMCID: PMC7754740 DOI: 10.1002/ehf2.12852] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/20/2020] [Revised: 05/20/2020] [Accepted: 06/02/2020] [Indexed: 11/29/2022] Open
Abstract
Aims Hypertrophic cardiomyopathy (HCM) is generally associated with mild disability and normal life expectancy. On the other hand, once the end‐stage phase of HCM characterized by left ventricular (LV) ejection fraction < 50% is established, patients with this subtype have a poor prognosis. This study clarifies the clinical parameters associated with progression to end‐stage HCM. Methods and results We retrospectively studied 157 HCM patients (age 59.9 ± 14.2 years, 104 men) with preserved LV systolic function in whom subsequent echocardiographic data were obtained for a period of >1 year. HCM progressed to end‐stage HCM in 13 patients (8.3%) of the 157 patients during a mean follow‐up period of 6.3 ± 2.8 years. Compared with patients who did not reach end‐stage HCM at the last evaluation, patients with progression to the end‐stage phase had lower ejection fraction, larger LV size, more enlarged left atrial diameter, longer follow‐up period, and higher frequency of an elevated concentration of high‐sensitivity cardiac troponin T (hs‐cTnT; >0.014 ng/mL) at registration. Multivariate analysis revealed that elevated hs‐cTnT was a significant predictor independent of lower LV ejection fraction for progression to end‐stage HCM. Furthermore, in patients with elevated hs‐cTnT levels, LV ejection fraction became significantly lower, LV end‐diastolic diameter increased, and LV wall thickness decreased during the follow‐up period, whereas those parameters did not change in the normal hs‐cTnT group. Conclusions In patients with HCM, an elevated hs‐cTnT was associated with progression of LV remodelling, and this biomarker can be useful for predicting progression to the end‐stage phase.
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Affiliation(s)
- Toru Kubo
- Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Kochi, Japan
| | - Yuri Ochi
- Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Kochi, Japan
| | - Yuichi Baba
- Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Kochi, Japan
| | - Kenta Sugiura
- Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Kochi, Japan
| | - Asa Takahashi
- Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Kochi, Japan
| | - Takayoshi Hirota
- Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Kochi, Japan
| | - Shigeo Yamanaka
- Department of Laboratory Medicine, Kochi Medical School, Kochi University, Kochi, Japan
| | - Naohito Yamasaki
- Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Kochi, Japan
| | - Yoshinori L Doi
- Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Kochi, Japan
| | - Hiroaki Kitaoka
- Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Kochi, Japan
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25
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Wu R, An DA, Shi RY, Chen BH, Wu CW, Jiang M, Xu JR, Wu LM, Pu J. The feasibility and diagnostic value of intravoxel incoherent motion diffusion-weighted imaging in the assessment of myocardial fibrosis in hypertrophic cardiomyopathy patients. Eur J Radiol 2020; 132:109333. [PMID: 33068839 DOI: 10.1016/j.ejrad.2020.109333] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/04/2019] [Revised: 02/27/2020] [Accepted: 09/30/2020] [Indexed: 10/23/2022]
Abstract
PURPOSE To investigate the feasibility and diagnostic value of intravoxel incoherent motion (IVIM) in the assessment of myocardial fibrosis in hypertrophic cardiomyopathy (HCM) patients. METHODS Fifty-five HCM patients underwent IVIM diffusion-weighted cardiovascular resonance imaging; Cine, T1 mapping, IVIM and late gadolinium enhancement (LGE) were performed. The relationship of strain, pre T1, extracellular volume (ECV), IVIM-derived parameters (D, D* and f) and LGE were analyzed based on 16 American Heart Association segments. Abnormal segments of myocardial fibrosis were defined as: the presence of LGE (LGE+) or ECV ≥ 29.6 %. RESULTS D parameter was significantly increased in LGE + vs LGE- (1.89 ± 0.14 μm2/ms vs. 1.63 ± 0.12 μm2/ms, p < 0.001) and ECV ≥ 29.6 % vs ECV < 29.6 % (1.84 ± 0.13 μm2/ms vs. 1.61 ± 0.12 μm2/ms, p < 0.001), respectively. D* and f parameters were significantly decreased in LGE + vs LGE- (D*: 34.9 ± 6.6 μm2/m vs 55.2 ± 11.4 μm2/m, p < 0.001; f: 10.8 ± 1.29 % vs 12.5 ± 1.26 %, p < 0.001) and ECV ≥ 29.6 % vs ECV < 29.6 % (D*: 37.5 ± 6.9 μm2/m vs 59.6 ± 9.2 μm2/m, p < 0.001; f: 10.9 ± 1.1 % vs 13.00 ± 1.0 %, p = 0.021), respectively. Moreover, significant correlations were demonstrated between D and ECV, as well as D* and f. CONCLUSIONS IVIM DW-CMR has proven to be ingenious in the investigation of myocardial fibrosis; D* and f parameters may have potential value to assess the perfusion status of fibrotic regions in HCM patients.
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Affiliation(s)
- Rui Wu
- Department of Radiology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, No.160 PuJian Road Shanghai 200127, China
| | - Dong-Aolei An
- Department of Radiology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, No.160 PuJian Road Shanghai 200127, China
| | - Ruo-Yang Shi
- Department of Radiology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, No.160 PuJian Road Shanghai 200127, China
| | - Bing-Hua Chen
- Department of Radiology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, No.160 PuJian Road Shanghai 200127, China
| | - Chong-Wen Wu
- Department of Radiology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, No.160 PuJian Road Shanghai 200127, China
| | - Meng Jiang
- Department of Cardiology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, No.160 PuJian Road Shanghai 200127, China
| | - Jian-Rong Xu
- Department of Radiology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, No.160 PuJian Road Shanghai 200127, China
| | - Lian-Ming Wu
- Department of Radiology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, No.160 PuJian Road Shanghai 200127, China.
| | - Jun Pu
- Department of Cardiology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, No.160 PuJian Road Shanghai 200127, China.
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Abstract
Cardiac magnetic resonance (CMR) imaging is an effective method for noninvasively imaging the heart which in the last two decades impressively enhanced spatial and temporal resolution and imaging speed, broadening its spectrum of applications in cardiovascular disease. CMR imaging techniques are designed to noninvasively assess cardiovascular morphology, ventricular function, myocardial perfusion, tissue characterization, flow quantification and coronary artery disease. These intrinsic features yield CMR suitable for diagnosis, follow-up and longitudinal monitoring after treatment of cardiovascular diseases. The aim of this paper is to review the technical basis of CMR, from cardiac imaging planes to cardiac imaging sequences.
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27
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Nakamura M, Kido T, Hirai K, Tabo K, Tanabe Y, Kawaguchi N, Kurata A, Kido T, Yamaguchi O, Mochizuki T. What is the mid-wall linear high intensity "lesion" on cardiovascular magnetic resonance late gadolinium enhancement? J Cardiovasc Magn Reson 2020; 22:66. [PMID: 32921308 PMCID: PMC7488664 DOI: 10.1186/s12968-020-00665-5] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/04/2020] [Accepted: 08/25/2020] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) is a valuable technique for detecting myocardial disorders and fibrosis. However, we sometimes observe a linear, mid-wall high intensity signal in the basal septum in the short axis view, which often presents diagnostic difficulties in the clinical setting. The purpose of this study was to compare the linear, mid-wall high intensity in the basal septum identified by LGE with the anterior septal perforator arteries identified by coronary computed tomography angiography (CorCTA). METHODS We retrospectively selected 148 patients who underwent both CorCTA and CMR LGE within 1 year. In the interpretation of LGE, we defined a positive linear high intensity (LHI+) as follows: ① LHI in the basal septum and ② observable for 1.5 cm or more. All other patients were defined as a negative LHI (LHI-). In LHI+ patients, we assessed the correlation between the LHI length and the septal perforator artery length on CorCTA. We also compared the length of the septal perforator artery on CorCTA between LHI+ patients and LHI- patients. RESULTS A population of 111 patients were used for further analysis. Among these , there were 55 LHI+ patients and 56 LHI- patients. In LHI+ patients, linear regression analysis revealed that there was a good agreement between LGE LHI and septal perforator arteries by CorCTA in terms of length measurements. The measured length of the anterior septal perforator arteries was significantly shorter in LHI- patients than in LHI+ patients (10 ± 8 mm vs. 21 ± 8 mm; P < 0.05). CONCLUSIONS The LHI observed in the basal septum on short axis LGE may reflect contrast enhancement of the anterior septal perforator arteries. It is important to interpret this septal LHI against knowledge of anatomic structure, to avoid misinterpretations of LGE and prevent misdiagnosis.
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Affiliation(s)
- Masashi Nakamura
- Department of Radiology, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime 791-0295 Japan
| | - Tomoyuki Kido
- Department of Radiology, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime 791-0295 Japan
| | - Kuniaki Hirai
- Department of Radiology, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime 791-0295 Japan
| | - Kohei Tabo
- Department of Radiology, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime 791-0295 Japan
| | - Yuki Tanabe
- Department of Radiology, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime 791-0295 Japan
| | - Naoto Kawaguchi
- Department of Radiology, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime 791-0295 Japan
| | - Akira Kurata
- Department of Radiology, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime 791-0295 Japan
| | - Teruhito Kido
- Department of Radiology, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime 791-0295 Japan
| | - Osamu Yamaguchi
- Department of Cardiology, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime 791-0295 Japan
| | - Teruhito Mochizuki
- Department of Radiology, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime 791-0295 Japan
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Updating the Risk Stratification for Sudden Cardiac Death in Cardiomyopathies: The Evolving Role of Cardiac Magnetic Resonance Imaging. An Approach for the Electrophysiologist. Diagnostics (Basel) 2020; 10:diagnostics10080541. [PMID: 32751773 PMCID: PMC7460122 DOI: 10.3390/diagnostics10080541] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/04/2020] [Revised: 07/27/2020] [Accepted: 07/28/2020] [Indexed: 12/11/2022] Open
Abstract
The prevention of sudden cardiac death (SCD) in cardiomyopathies (CM) remains a challenge. The current guidelines still favor the implantation of devices for the primary prevention of SCD only in patients with severely reduced left ventricular ejection fraction (LVEF) and heart failure (HF) symptoms. The implantation of an implantable cardioverter-defibrillator (ICD) is a protective barrier against arrhythmic events in CMs, but the benefit does not outweigh the cost in low risk patients. The identification of high risk patients is the key to an individualized prevention strategy. Cardiac magnetic resonance (CMR) provides reliable and reproducible information about biventricular function and tissue characterization. Furthermore, late gadolinium enhancement (LGE) quantification and pattern of distribution, as well as abnormal T1 mapping and extracellular volume (ECV), representing indices of diffuse fibrosis, can enhance our ability to detect high risk patients. CMR can also complement electro-anatomical mapping (EAM), a technique already applied in the risk evaluation and in the ventricular arrhythmias ablation therapy of CM patients, providing a more accurate assessment of fibrosis and arrhythmic corridors. As a result, CMR provides a new insight into the pathological substrate of CM. CMR may help identify high risk CM patients and, combined with EAM, can provide an integrated evaluation of scar and arrhythmic corridors in the ablative therapy of ventricular arrhythmias.
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29
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Aquaro GD, Grigoratos C, Bracco A, Proclemer A, Todiere G, Martini N, Habtemicael YG, Carerj S, Sinagra G, Di Bella G. Late Gadolinium Enhancement-Dispersion Mapping: A New Magnetic Resonance Imaging Technique to Assess Prognosis in Patients With Hypertrophic Cardiomyopathy and Low-Intermediate 5-Year Risk of Sudden Death. Circ Cardiovasc Imaging 2020; 13:e010489. [PMID: 32539460 DOI: 10.1161/circimaging.120.010489] [Citation(s) in RCA: 15] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
BACKGROUND Late gadolinium enhancement (LGE) is an important prognostic marker in hypertrophic cardiomyopathy and an extent >15% it is associated with high risk of sudden cardiac death. We proposed a novel method, the LGE-dispersion mapping, to assess heterogeneity of scar, and evaluated its prognostic role in patients with hypertrophic cardiomyopathy. METHODS One hundred eighty-three patients with hypertrophic cardiomyopathy and a low- or intermediate 5-year risk of sudden cardiac death underwent cardiac magnetic resonance imaging. A parametric map was generated from each LGE image. A score from 0 to 8 was assigned at every pixel of these maps, indicating the number of the surrounding pixels having different quality (nonenhancement, mild-enhancement, or hyperenhancement) from the central pixel. The Global Dispersion Score (GDS) was calculated as the average score of all the pixels of the images. RESULTS During a median follow-up time of 6 (25th-75th, 4-10) years, 22 patients had hard cardiac events (sudden cardiac death, appropriate implantable cardioverter-defibrillator therapy, resuscitated cardiac arrest, and sustained ventricular tachycardia). Kaplan-Meier analysis showed that patients with GDS>0.86 had worse prognosis than those with lower GDS (P<0.0001). GDS>0.86 was the only independent predictor of cardiac events (hazard ratio, 9.9 [95% CI, 2.9-34.6], P=0.0003). When compared with LGE extent >15%, GDS improved the classification of risk in these patients (net reclassification improvement, 0.39 [95% CI, 0.11-0.72], P<0.019). CONCLUSIONS LGE-dispersion mapping is a marker of scar heterogeneity and provides a better risk stratification than LGE presence and its extent in patients with hypertrophic cardiomyopathy and a low-intermediate 5-year risk of sudden cardiac death.
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Affiliation(s)
| | | | - Antonio Bracco
- Department of Cardiology, University of Messina, Messina, Italy (A.B., S.C., G.D.B.)
| | - Alberto Proclemer
- Cardio-thoraco-vascular Department, University of Trieste, Trieste, Italy (A.P., G.S.)
| | - Giancarlo Todiere
- Fondazione Toscana G. Monasterio, Pisa, Italy (G.D.A., C.G., G.T., N.M.)
| | - Nicola Martini
- Fondazione Toscana G. Monasterio, Pisa, Italy (G.D.A., C.G., G.T., N.M.)
| | | | - Scipione Carerj
- Department of Cardiology, University of Messina, Messina, Italy (A.B., S.C., G.D.B.)
| | - Gianfranco Sinagra
- Cardio-thoraco-vascular Department, University of Trieste, Trieste, Italy (A.P., G.S.)
| | - Gianluca Di Bella
- Department of Cardiology, University of Messina, Messina, Italy (A.B., S.C., G.D.B.)
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Differentiating Athlete's Heart from Left Ventricle Cardiomyopathies. J Cardiovasc Transl Res 2020; 13:265-273. [PMID: 32410209 DOI: 10.1007/s12265-020-10021-8] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/02/2019] [Accepted: 04/28/2020] [Indexed: 01/05/2023]
Abstract
Imaging techniques have allowed knowing the structural adaptative changes observed in the hearts of highly trained athletes. Athletes can develop very marked structural changes and the need may rise for a differential diagnosis with real cardiomyopathy. In this chapter, authors review the physiologic and morphologic features associated with athletic training and the keys to differentiate normal adaptive athlete's heart from mild or initial expression forms of left-heart side cardiomyopathies such as hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), and left ventricle non-compaction (LVNC).
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31
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Kim EK, Lee SC, Chang SA, Jang SY, Kim SM, Park SJ, Choi JO, Park SW, Jeon ES, Choe YH. Prevalence and clinical significance of cardiovascular magnetic resonance adenosine stress-induced myocardial perfusion defect in hypertrophic cardiomyopathy. J Cardiovasc Magn Reson 2020; 22:30. [PMID: 32366254 PMCID: PMC7199346 DOI: 10.1186/s12968-020-00623-1] [Citation(s) in RCA: 24] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/07/2019] [Accepted: 04/07/2020] [Indexed: 11/23/2022] Open
Abstract
BACKGROUND Hypertrophic cardiomyopathy (HCM) is thought to be associated with microvascular dysfunction. Adenosine stress-perfusion cardiovascular magnetic resonance imaging (CMR) is a sensitive method for assessing microvascular perfusion abnormalities. We evaluated the prevalence and clinical characteristics of HCM patients with adenosine-induced perfusion defects on CMR. METHODS Among 189 consecutive patients with HCM who underwent adenosine-stress perfusion CMR, 115 patients who had clinical, echocardiography, 24-h Holter monitoring and treadmill exercise test data were analyzed. We calculated myocardial perfusion ratio index from the intensity-over-time curve to quantify perfusion defects. The presence and extent of the stress-induced perfusion defect were compared with clinical characteristics, presence and extent of late gadolinium enhancement (LGE), left ventricular (LV) mass index and volume, presence of non-sustained ventricular tachycardia (NSVT) and results of treadmill exercise test. RESULTS The mean age of enrolled patients was 51.8 ± 11.3 years. Most patients were asymptomatic except 25 subjects presented with New York Heart Association Class II dyspnea and 16 patients with atypical non-exertional chest discomfort. LGE was present in 103 (89.6%) subjects. Adenosine stress-induced perfusion defects were present in 48 (42%) subjects. None of the perfusion defects corresponded with a single or multiple coronary artery territories, showing a multiple patchy pattern in 24 (50.0%), a concentric subendocardial pattern in 20 subjects (41.7%), and as a single blot-like defect in the remaining 4 (8.3%). A perfusion defect was associated with NSVT, LV apical aneurysm, higher LV mass index, and higher LGE volume on univariate analysis. Multivariate analysis revealed female gender (P = 0.008), presence of apical aneurysm and NSVT (P = 0.036 and 0.047, respectively), and LV mass index (P = 0.022) to be independently associated with adenosine stress-induced perfusion defects. CONCLUSIONS In patients with HCM, adenosine-stress perfusion defects on CMR are present in more than 40% of subjects. This perfusion defect is associated with NSVT, higher LV mass index, and apical aneurysms. The prognostic value of this finding needs further elucidation.
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Affiliation(s)
- Eun Kyoung Kim
- Division of Cardiology, Department of Medicine, Samsung Medical Center, Heart Vascular Stroke Institute, Sungkyunkwan University School of Medicine, #81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea
| | - Sang-Chol Lee
- Division of Cardiology, Department of Medicine, Samsung Medical Center, Heart Vascular Stroke Institute, Sungkyunkwan University School of Medicine, #81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea.
| | - Sung-A Chang
- Division of Cardiology, Department of Medicine, Samsung Medical Center, Heart Vascular Stroke Institute, Sungkyunkwan University School of Medicine, #81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea
| | - Shin-Yi Jang
- Division of Cardiology, Department of Medicine, Samsung Medical Center, Heart Vascular Stroke Institute, Sungkyunkwan University School of Medicine, #81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea
| | - Sung Mok Kim
- Department of Radiology and Cardiovascular Imaging Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea
| | - Sung-Ji Park
- Division of Cardiology, Department of Medicine, Samsung Medical Center, Heart Vascular Stroke Institute, Sungkyunkwan University School of Medicine, #81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea
| | - Jin-Oh Choi
- Division of Cardiology, Department of Medicine, Samsung Medical Center, Heart Vascular Stroke Institute, Sungkyunkwan University School of Medicine, #81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea
| | - Seung Woo Park
- Division of Cardiology, Department of Medicine, Samsung Medical Center, Heart Vascular Stroke Institute, Sungkyunkwan University School of Medicine, #81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea
| | - Eun-Seok Jeon
- Division of Cardiology, Department of Medicine, Samsung Medical Center, Heart Vascular Stroke Institute, Sungkyunkwan University School of Medicine, #81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea
| | - Yeon Hyeon Choe
- Department of Radiology and Cardiovascular Imaging Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea.
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Habert P, Capron T, Hubert S, Bentatou Z, Bartoli A, Tradi F, Renard S, Rapacchi S, Guye M, Bernard M, Habib G, Jacquier A. Quantification of right ventricular extracellular volume in pulmonary hypertension using cardiac magnetic resonance imaging. Diagn Interv Imaging 2020; 101:311-320. [DOI: 10.1016/j.diii.2019.12.008] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/04/2019] [Revised: 12/09/2019] [Accepted: 12/12/2019] [Indexed: 12/30/2022]
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Antunes MDO, Scudeler TL. Hypertrophic cardiomyopathy. IJC HEART & VASCULATURE 2020; 27:100503. [PMID: 32309534 PMCID: PMC7154317 DOI: 10.1016/j.ijcha.2020.100503] [Citation(s) in RCA: 21] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/07/2020] [Revised: 03/13/2020] [Accepted: 03/17/2020] [Indexed: 02/07/2023]
Abstract
Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disease. The disease is characterized by marked variability in morphological expression and natural history, ranging from asymptomatic to heart failure or sudden cardiac death. Left ventricular hypertrophy and abnormal ventricular configuration result in dynamic left ventricular outflow obstruction in most patients. The goal of pharmacological therapy in HCM is to alleviate the symptoms, and it includes pharmacotherapies and septal reduction therapies. In this review, we summarize the relevant clinical issues and treatment options of HCM.
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Affiliation(s)
- Murillo de Oliveira Antunes
- Instituto do Coração (InCor), Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil
- Universidade São Francisco (USF), Bragança Paulista, São Paulo, Brazil
| | - Thiago Luis Scudeler
- Instituto do Coração (InCor), Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil
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Kamenický P, Maione L, Chanson P. Cardiovascular complications of acromegaly. ANNALES D'ENDOCRINOLOGIE 2020; 82:206-209. [PMID: 33168155 DOI: 10.1016/j.ando.2020.03.010] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 10/24/2022]
Abstract
Acromegaly is a chronic disease due to growth hormone (GH) and insulin-like growth factor 1 (IGF-1) excess. It is associated with various systemic complications including cardiovascular disease. Arterial hypertension occurs in about 20% to 30% of patients. Its pathogenesis is mainly related to the increase in plasma volume secondary to a sodium retaining actions of GH and IGF-1 in the kidney, but abnormalities in vessel architecture and reactivity participate. Left ventricular hypertrophy and diastolic dysfunctions were frequently reported in echo-based studies and are mostly mild and without clinical consequences. Recent cardiac MRI studies described a much lower frequency of myocardial hypertrophy than echo-based assessments. Progression to systolic dysfunction with congestive heart failure is nowadays very rare. Risk of coronary heart disease and of clinically significant arrythmias does not seem to be increased. Acromegaly-related cardiac valve abnormalities may be related to fibrotic changes and seem to persist after effective treatment of acromegaly. Advances in acromegaly treatment over the last decades significantly diminished the cardiovascular burden of the disease, with the cardiovascular disease anymore being the leading cause of death.
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Affiliation(s)
- Peter Kamenický
- Assistance Publique-Hôpitaux de Paris, Hôpital de Bicêtre, Service d'Endocrinologie et des Maladies de la Reproduction, Centre de Référence des Maladies Rares de l'Hypophyse (HYPO), Université Paris-Saclay, Inserm, Physiologie et Physiopathologie Endocriniennes, Le Kremlin-Bicêtre, France.
| | - Luigi Maione
- Assistance Publique-Hôpitaux de Paris, Hôpital de Bicêtre, Service d'Endocrinologie et des Maladies de la Reproduction, Centre de Référence des Maladies Rares de l'Hypophyse (HYPO), Université Paris-Saclay, Inserm, Physiologie et Physiopathologie Endocriniennes, Le Kremlin-Bicêtre, France
| | - Philippe Chanson
- Assistance Publique-Hôpitaux de Paris, Hôpital de Bicêtre, Service d'Endocrinologie et des Maladies de la Reproduction, Centre de Référence des Maladies Rares de l'Hypophyse (HYPO), Université Paris-Saclay, Inserm, Physiologie et Physiopathologie Endocriniennes, Le Kremlin-Bicêtre, France
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Gadabanahalli K, Bhat V, Kanagasabai K, Rao PV. Magnetic Resonance Imaging Assessment of Pre- and Post-Surgery Myocardial Changes in Hypertrophic Cardiomyopathy: Correlation with Echocardiography. J Clin Imaging Sci 2020; 10:4. [PMID: 32123618 PMCID: PMC7049884 DOI: 10.25259/jcis_162_2019] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/15/2020] [Accepted: 01/22/2020] [Indexed: 11/25/2022] Open
Abstract
Hypertrophic cardiomyopathy (HCM) is a common form of cardiomyopathy and a leading cause of sudden death in the young. Magnetic resonance imaging (MRI) is an established pre-operative tool for the evaluating of patients suspected with HCM for morphological assessment and identifying patients at risk of sudden death. Echocardiography and MRI are equally used in the post-treatment assessment of cardiac function and morphology. In this report, we present the comparative role of these two modalities in pre- and post-operative imaging assessment in our patients, treated surgically with the left ventricular myomectomy. Relative merits of MRI and echocardiography are presented and discussed.
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Affiliation(s)
- Karthik Gadabanahalli
- Departments of Radiology, Narayana Health, Narayana Hrudayalaya Hospital, Bengaluru, India
| | - Venkatraman Bhat
- Departments of Radiology, Narayana Health, Narayana Hrudayalaya Hospital, Bengaluru, India
| | - K Kanagasabai
- Departments of Radiology, Narayana Health, Narayana Hrudayalaya Hospital, Bengaluru, India
| | - P V Rao
- Departments of Cardiac surgery, Narayana Health, Narayana Hrudayalaya Hospital, Bengaluru, India
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Caruso MR, Garg L, Martinez MW. Cardiac Imaging in the Athlete: Shrinking the "Gray Zone". CURRENT TREATMENT OPTIONS IN CARDIOVASCULAR MEDICINE 2020; 22:5. [PMID: 32016641 DOI: 10.1007/s11936-020-0802-8] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/02/2023]
Abstract
PURPOSE OF THE REVIEW This review will explore frequently encountered diagnostic challenges and summarize the role cardiac imaging plays in defining the boundaries of what constitutes the athlete's heart syndrome versus pathology. RECENT FINDINGS Investigations have predominantly focused on differentiating the athlete's heart from potentially lethal pathological conditions that may produce a similar cardiac morphology. Guidelines have identified criteria for identifying definitive pathology, but difficulty arises when individuals fall in the gray zone of expected athletic remodeling and pathology. Transthoracic echo has traditionally been the imaging modality of choice utilizing parameters such as wall thickness, wall:volume ratio, and certain diastolic parameters. Newer echocardiogram techniques such as strain imaging and speckle tracking have potential additive utility but still need further investigation. Cardiac magnetic resonance (CMR) imaging has emerged as an additive technique to help differentiate the phenotypic overlap between these groups. Utilizing gadolinium enhancement and T1 mapping along with its excellent spatial resolution can help distinguish pathology from physiology. Both established and novel cardiac imaging modalities have been used for uncovering the at risk athletes with cardiomyopathies. The issue is of practical importance because athletes are frequently referred to the cardiologist with symptoms of fatigue, palpitations, presyncope, and/or syncope concerned about the safety of their future participation. Imaging is a key component of risk stratification and identifying normal findings of the developed athlete and those "at-risk" athletes.
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Affiliation(s)
- Mario R Caruso
- Department of Cardiovascular Medicine, Lehigh Valley Health Network, Allentown, PA, 18103, USA
| | - Lohit Garg
- Department of Cardiovascular Medicine, Lehigh Valley Health Network, Allentown, PA, 18103, USA
| | - Matthew W Martinez
- Department of Cardiovascular Medicine, Atlantic Health, Morristown Medical Center, Morristown, NJ, 07960, USA. .,Sports Cardiology and Hypertrophic Cardiomyopathy, 111 S Madison Ave, Suite 300, Morristown, NJ, 07960, USA.
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Sagawa Y, Nagata Y, Yamaguchi T, Mitsui K, Nagamine T, Yamaguchi J, Hijikata S, Watanabe K, Masuda R, Miyazaki R, Kaneko M, Miwa N, Sekigawa M, Hara N, Nozato T, Ashikaga T, Goya M, Sasano T, Hirao K. Long-Term Performance of Right Ventricular Implantable Cardioverter-Defibrillator Leads in Arrhythmogenic Right Ventricular Cardiomyopathy and Hypertrophic Cardiomyopathy. Int Heart J 2020; 61:39-45. [PMID: 31956141 DOI: 10.1536/ihj.19-279] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/18/2022]
Abstract
Patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) and hypertrophic cardiomyopathy (HCM) implanted with implantable cardioverter-defibrillators (ICDs) may show a large decrease in R-wave amplitude during long-term follow-up. However, it is unclear whether this decrease is higher in these patients than in those without structural heart disease. This study investigated ICD-lead intracardiac parameters over a long duration in patients with ARVC and HCM and compared these parameters with those of a control group. We included 50 patients (mean age, 55.2 ± 17.2 years; 26% female) with ICD leads in the right ventricular apex, and compared 7 ARVC and 14 HCM patients with 29 control patients without structural heart disease. ICD-lead parameters, including R-wave amplitude, pacing threshold, and impedance during follow-up, were compared. The difference in these parameters between the time of implantation and year 5 were also compared. There were no significant differences in R-wave amplitude at implantation among the 3 groups. The change in R-wave amplitude between the time of implantation and year 5 was significantly greater in the ARVC group (-3.3 ± 5.4 mV, P = 0.012) in comparison to the control group (1.3 ± 2.8 mV); the HCM group showed no significant difference (-0.4 ± 2.3 mV, P = 0.06). Thus, in the ARVC group, R-wave amplitude at year 5 was significantly lower than that in the control group (5.7 ± 4.8 mV versus 12.5 ± 4.5 mV, P = 0.001). In ARVC patients with ICDs, ventricular sensing is likely to deteriorate during long-term follow-up; however, in HCM patients, sensing may not deteriorate.
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Affiliation(s)
- Yuichiro Sagawa
- Department of Cardiology, Japanese Red Cross Musashino Hospital
| | | | | | - Kentaro Mitsui
- Department of Cardiology, Japanese Red Cross Musashino Hospital
| | | | - Junji Yamaguchi
- Department of Cardiology, Japanese Red Cross Musashino Hospital
| | | | - Keita Watanabe
- Department of Cardiology, Japanese Red Cross Musashino Hospital
| | - Ryo Masuda
- Department of Cardiology, Japanese Red Cross Musashino Hospital
| | | | - Masakazu Kaneko
- Department of Cardiology, Japanese Red Cross Musashino Hospital
| | - Naoyuki Miwa
- Department of Cardiology, Japanese Red Cross Musashino Hospital
| | | | - Nobuhiro Hara
- Department of Cardiology, Japanese Red Cross Musashino Hospital
| | | | | | - Masahiko Goya
- Department of Cardiovascular Medicine, Tokyo Medical and Dental University
| | - Tetsuo Sasano
- Department of Cardiovascular Medicine, Tokyo Medical and Dental University
| | - Kenzo Hirao
- Arrhythmia Advanced Therapy Center, AOI Universal Hospital
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Badran HM, Soltan G, Almeleigi R, Faheem N, Yacoub MH. Prognostic significance of left ventricular end diastolic pressure using E/E' in patients with hypertrophic cardiomyopathy. Echocardiography 2019; 36:2167-2175. [PMID: 31742769 DOI: 10.1111/echo.14539] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/24/2019] [Revised: 09/27/2019] [Accepted: 10/25/2019] [Indexed: 11/27/2022] Open
Abstract
BACKGROUND Left ventricular (LV) diastolic dysfunction is a prominent feature of hypertrophic cardiomyopathy (HCM). Prediction of LV filling pressure using the ratio between early diastolic transmitral flow and mitral annular velocity (E/e') had proved a good accuracy. AIM OF THIS STUDY We investigated the value of E/e' to predict cardiovascular (CV) mortality in patients with HCM. METHODS A total of 243 patients with HCM had E/e' measured in combination with clinical evaluation, conventional echocardiographic measurements, cardiopulmonary exercise evaluation, and Holter monitoring. RESULTS During a mean follow-up of (3.2 ± 1.2 years), 17 (7%) patients died. Non survivors had significantly higher SBP, DBP, left ventricular outflow tract obstruction (LVOTO) gradient, mitral E, and E/e', but lower e' of mitral annulus and more prevalent restrictive filling pattern. E/e' was directly correlated with age (r = .24, P < .005), left atrial volume index (r = .44, P < .0001), LVMI (r=0.23,P<.005), LVOT gradient (r = .43, P < .0001), NYHA class (r = .19, P < .006), pulmonary artery pressure (r = .24, P < .005), positive family history of HCM (r = .22, P < .005), and inversely related to peak systolic velocity (S) (r = .44, P < .0001). By multivariate analysis, only LVOTO ([RR] 4.11, 95% CI 1.002 to 1.148, P < .04) and E/e' were independent predictors for overall mortality in HCM (relative risk [RR] 5.27, 95% CI 1.002 to 1.024, P < .02). The risk of dying increased with increasing E/e' ratio, being approximately 4 times higher for patients in the highest quartile (HR 3.8 (CI 1.38-5.12, log-rank < 0.002)). CONCLUSIONS In hypertrophic cardiomyopathy, the E/e' ratio remains a powerful predictor of all-cause mortality, particularly if it is associated with LVOT obstruction.
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Affiliation(s)
- Hala Mahfouz Badran
- Cardiology Department, Menoufia University, Tanta, Egypt.,The BAHCM National Program, Tanta, Egypt
| | - Ghada Soltan
- Cardiology Department, Menoufia University, Tanta, Egypt
| | - Reda Almeleigi
- Cardiology Department, Menoufia University, Tanta, Egypt
| | - Naglaa Faheem
- Cardiology Department, Menoufia University, Tanta, Egypt.,The BAHCM National Program, Tanta, Egypt
| | - Magdi H Yacoub
- The BAHCM National Program, Tanta, Egypt.,Imperial College, London, UK
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Torres-Matiz JA, Carvajal-Rivera JJ. Diagnóstico y estudio de cardiopatías infrecuentes: multimodalidad – miocardiopatía hipertrófica. REVISTA COLOMBIANA DE CARDIOLOGÍA 2019. [DOI: 10.1016/j.rccar.2019.05.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/10/2023] Open
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Miller RJH, Heidary S, Pavlovic A, Schlachter A, Dash R, Fleischmann D, Ashley EA, Wheeler MT, Yang PC. Defining genotype-phenotype relationships in patients with hypertrophic cardiomyopathy using cardiovascular magnetic resonance imaging. PLoS One 2019; 14:e0217612. [PMID: 31199839 PMCID: PMC6568393 DOI: 10.1371/journal.pone.0217612] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/04/2019] [Accepted: 05/16/2019] [Indexed: 01/28/2023] Open
Abstract
PURPOSE HCM is the most common inherited cardiomyopathy. Historically, there has been poor correlation between genotype and phenotype. However, CMR has the potential to more accurately assess disease phenotype. We characterized phenotype with CMR in a cohort of patients with confirmed HCM and high prevalence of genetic testing. METHODS Patients with a diagnosis of HCM, who had undergone contrast-enhanced CMR were identified. Left ventricular mass index (LVMI) and volumes were measured from steady-state free precession sequences. Late gadolinium enhancement (LGE) was quantified using the full width, half maximum method. All patients were prospectively followed for the development of septal reduction therapy, arrhythmia or death. RESULTS We included 273 patients, mean age 51.2 ± 15.5, 62.9% male. Of those patients 202 (74.0%) underwent genetic testing with 90 pathogenic, likely pathogenic, or rare variants and 13 variants of uncertain significance identified. Median follow-up was 1138 days. Mean LVMI was 82.7 ± 30.6 and 145 patients had late gadolinium enhancement (LGE). Patients with beta-myosin heavy chain (MYH7) mutations had higher LV ejection fraction (68.8 vs 59.1, p<0.001) than those with cardiac myosin binding protein C (MYBPC3) mutations. Patients with MYBPC3 mutations were more likely to have LVEF < 55% (29.7% vs 4.9%, p = 0.005) or receive a defibrillator than those with MYH7 mutations (54.1% vs 26.8%, p = 0.020). CONCLUSIONS We found that patients with MYBPC3 mutations were more likely to have impaired ventricular function and may be more prone to arrhythmic events. Larger studies using CMR phenotyping may be capable of identifying additional characteristics associated with less frequent genetic causes of HCM.
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Affiliation(s)
- Robert J. H. Miller
- Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, California, United States of America
| | - Shahriar Heidary
- Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, California, United States of America
| | - Aleksandra Pavlovic
- Center for Inherited Cardiovascular Disease, Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, California, United States of America
| | - Audrey Schlachter
- Center for Inherited Cardiovascular Disease, Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, California, United States of America
| | - Rajesh Dash
- Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, California, United States of America
| | - Dominik Fleischmann
- Department of Radiology, Stanford University School of Medicine, Stanford, California, United States of America
| | - Euan A. Ashley
- Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, California, United States of America
- Center for Inherited Cardiovascular Disease, Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, California, United States of America
| | - Matthew T. Wheeler
- Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, California, United States of America
- Center for Inherited Cardiovascular Disease, Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, California, United States of America
| | - Phillip C. Yang
- Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, California, United States of America
- * E-mail:
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Zhou J, Zhou Y, Wang CX. LncRNA-MIAT regulates fibrosis in hypertrophic cardiomyopathy (HCM) by mediating the expression of miR-29a-3p. J Cell Biochem 2019; 120:7265-7275. [PMID: 30548303 DOI: 10.1002/jcb.28001] [Citation(s) in RCA: 17] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2018] [Accepted: 10/08/2018] [Indexed: 01/24/2023]
Abstract
AIM This study aimed to investigate the molecular mechanism underlying the fibrosis in hypertrophic cardiomyopathy (HCM). METHOD Quantitative real-time polymerase chain reaction (qRT-PCR) was performed to measure the expression of potentially relevant microRNAs (miRNAs) and long noncoding RNAs (lncRNAs) in patients with HCM suffering from fibrosis and patients with HCM free of fibrosis. In addition, the regulatory relationship between lncRNAs and miR-29a was studied using a luciferase assay. Subsequently, area under the receiver-operating characteristics (ROC) curve (AUC) analysis was conducted to predict the diagnostic value of myocardial infarction-associated transcript (MIAT), miR-29a, H19, and MEG3 in patients with HCM. Finally, the predicted regulatory relationship betwe en miR-29a and MIAT was validated by transfecting cells with different plasmids. RESULT miR-29a and MIAT were differently expressed between the fibrosis (+) HCM group and the fibrosis (-) HCM group, thus establishing a negative relationship between the expression of these two genes. In addition, both MIAT and miR-29a showed the ability to accurately predict the prognosis in patients with HCM. Furthermore, the luciferase activity of wild-type MIAT was evidently suppressed in cells transfected with miR-29a mimics, suggesting that the expression of miR-29a was apparently downregulated in the presence of MIAT. CONCLUSION The results obtained in this study collectively indicated that the MIAT might be associated with the development of fibrosis (+) HCM via negatively regulating the expression of miR-29a.
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Affiliation(s)
- Jing Zhou
- Cardiology Department, The Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, China
- Cardiology Department, Yan'an University Affiliated Hospital, Yan'an, Shaanxi, China
| | - Yu Zhou
- Nursing Department, The People's Hospital of Baoji, Baoji, Shaanxi, China
| | - Cong-Xia Wang
- Cardiology Department, The Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, China
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Abstract
Heart failure is a clinical syndrome with a broad spectrum of presentations. Cardiovascular imaging techniques such as echocardiography, cardiovascular magnetic resonance, computed tomography, and nuclear imaging play a crucial role in diagnosis, guiding management, and providing prognostic information. Each of these imaging modalities has their own respective strengths and weaknesses. Cardiac imaging can help differentiate between ischemic and nonischemic cardiomyopathies. Additionally, imaging techniques can display disease-specific findings, aiding in diagnosis of nonischemic cardiomyopathies and can provide a means to monitor response to therapy. The choice of imaging modality in the workup of cardiomyopathy should be based on the specific clinical question and the knowledge of the strengths and limitations of each imaging modality.
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Affiliation(s)
- Kate Rankin
- Division of Cardiology, Peter Munk Cardiac Center, Ted Rogers Program in Cardiotoxicity Prevention, Toronto General Hospital, University Health Network, University of Toronto, 4N-490, 585 University Avenue, Toronto, Ontario M5G 2N2, Canada
| | - Babitha Thampinathan
- Division of Cardiology, Peter Munk Cardiac Center, Ted Rogers Program in Cardiotoxicity Prevention, Toronto General Hospital, University Health Network, University of Toronto, 4N-490, 585 University Avenue, Toronto, Ontario M5G 2N2, Canada
| | - Paaladinesh Thavendiranathan
- Division of Cardiology, Peter Munk Cardiac Center, Ted Rogers Program in Cardiotoxicity Prevention, Toronto General Hospital, University Health Network, University of Toronto, 4N-490, 585 University Avenue, Toronto, Ontario M5G 2N2, Canada.
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Parikh VN, Caleshu C, Reuter C, Lazzeroni LC, Ingles J, Garcia J, McCaleb K, Adesiyun T, Sedaghat-Hamedani F, Kumar S, Graw S, Gigli M, Stolfo D, Ferro MD, Ing AY, Nussbaum R, Funke B, Wheeler MT, Hershberger RE, Cook S, Steinmetz L, Lakdawala NK, Taylor MRG, Mestroni L, Merlo M, Sinagra G, Semsarian C, Meder B, Judge DP, Ashley EA. Regional Variation in RBM20 Causes a Highly Penetrant Arrhythmogenic Cardiomyopathy. Circ Heart Fail 2019; 12:e005371. [PMID: 30871351 PMCID: PMC6422044 DOI: 10.1161/circheartfailure.118.005371] [Citation(s) in RCA: 94] [Impact Index Per Article: 15.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/23/2018] [Accepted: 12/28/2018] [Indexed: 12/26/2022]
Abstract
Background Variants in the cardiomyocyte-specific RNA splicing factor RBM20 have been linked to familial cardiomyopathy, but the causative genetic architecture and clinical consequences of this disease are incompletely defined. Methods and Results To define the genetic architecture of RBM20 cardiomyopathy, we first established a database of RBM20 variants associated with cardiomyopathy and compared these to variants observed in the general population with respect to their location in the RBM20 coding transcript. We identified 2 regions significantly enriched for cardiomyopathy-associated variants in exons 9 and 11. We then assembled a registry of 74 patients with RBM20 variants from 8 institutions across the world (44 index cases and 30 from cascade testing). This RBM20 patient registry revealed highly prevalent family history of sudden cardiac death (51%) and cardiomyopathy (72%) among index cases and a high prevalence of composite arrhythmias (including atrial fibrillation, nonsustained ventricular tachycardia, implantable cardiac defibrillator discharge, and sudden cardiac arrest, 43%). Patients harboring variants in cardiomyopathy-enriched regions identified by our variant database analysis were enriched for these findings. Further, these characteristics were more prevalent in the RBM20 registry than in large cohorts of patients with dilated cardiomyopathy and TTNtv cardiomyopathy and not significantly different from a cohort of patients with LMNA-associated cardiomyopathy. Conclusions Our data establish RBM20 cardiomyopathy as a highly penetrant and arrhythmogenic cardiomyopathy. These findings underline the importance of arrhythmia surveillance and family screening in this disease and represent the first step in defining the genetic architecture of RBM20 disease causality on a population level.
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Affiliation(s)
- Victoria N. Parikh
- Division of Cardiovascular Medicine, Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA
| | - Colleen Caleshu
- Division of Cardiovascular Medicine, Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA
| | - Chloe Reuter
- Division of Cardiovascular Medicine, Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA
| | - Laura C. Lazzeroni
- Depts. Of Psychiatry and Behavioral Sciences and of Biomedical Data Science, Stanford University School of Medicine, Stanford, CA, USA
| | - Jodie Ingles
- Department of Cardiology, Royal Prince Alfred Hospital and Agnes Ginges Centre for Molecular Cardiology, Centenary Institute, University of Sydney, NSW, Australia
| | | | | | | | - Farbod Sedaghat-Hamedani
- Institute for Cardiomyopathies, University Hospital Heidelberg, German Center for Cardiovascular Research (DZHK)
| | - Saurabh Kumar
- Brigham and Women’s Hospital, Partners Health Care and Harvard Medical School, Boston, MA, USA
| | - Sharon Graw
- Cardiovascular Institute, University of Colorado Anschutz Medical Campus, Aurora, CO, USA
| | - Marta Gigli
- Cardiovascular Department, Azienda Sanitaria Universitaria Integrata (ASUITS) and University of Trieste, Trieste, Italy
| | - Davide Stolfo
- Cardiovascular Department, Azienda Sanitaria Universitaria Integrata (ASUITS) and University of Trieste, Trieste, Italy
| | - Matteo Dal Ferro
- Cardiovascular Department, Azienda Sanitaria Universitaria Integrata (ASUITS) and University of Trieste, Trieste, Italy
| | - Alexander Y. Ing
- Laboratory of Molecular Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA
| | | | - Birgit Funke
- Laboratory of Molecular Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA
| | - Matthew T. Wheeler
- Division of Cardiovascular Medicine, Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA
| | - Ray E. Hershberger
- Divisions of Human Genetics and Cardiovascular Medicine, Department of Medicine, The Ohio State University College of Medicine, Columbus, OH
| | - Stuart Cook
- National Heart Lung Institute, Imperial College London, UK and National Heart Centre, Singapore
| | - Lars Steinmetz
- Department of Genetics, Stanford University School of Medicine, Stanford, CA,USA
| | - Neal K. Lakdawala
- Brigham and Women’s Hospital, Partners Health Care and Harvard Medical School, Boston, MA, USA
| | - Matthew RG Taylor
- Cardiovascular Institute, University of Colorado Anschutz Medical Campus, Aurora, CO, USA
| | - Luisa Mestroni
- Cardiovascular Institute, University of Colorado Anschutz Medical Campus, Aurora, CO, USA
| | - Marco Merlo
- Cardiovascular Department, Azienda Sanitaria Universitaria Integrata (ASUITS) and University of Trieste, Trieste, Italy
| | - Gianfranco Sinagra
- Cardiovascular Department, Azienda Sanitaria Universitaria Integrata (ASUITS) and University of Trieste, Trieste, Italy
| | - Christopher Semsarian
- Department of Cardiology, Royal Prince Alfred Hospital and Agnes Ginges Centre for Molecular Cardiology, Centenary Institute, University of Sydney, NSW, Australia
| | - Benjamin Meder
- Institute for Cardiomyopathies, University Hospital Heidelberg, German Center for Cardiovascular Research (DZHK)
- Department of Genetics, Stanford University School of Medicine, Stanford, CA,USA
| | - Daniel P. Judge
- Department of Medicine, Medical University of South Carolina, Charleston, SC, USA
| | - Euan A. Ashley
- Division of Cardiovascular Medicine, Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA
- Department of Genetics, Stanford University School of Medicine, Stanford, CA,USA
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Microvascular Dysfunction in Hypertrophic Cardiomyopathy. CURRENT CARDIOVASCULAR IMAGING REPORTS 2019. [DOI: 10.1007/s12410-019-9478-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/10/2023]
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Yi JE, Park J, Lee HJ, Shin DG, Kim Y, Kim M, Kwon K, Pyun WB, Kim YJ, Joung B. Prognostic implications of late gadolinium enhancement at the right ventricular insertion point in patients with non-ischemic dilated cardiomyopathy: A multicenter retrospective cohort study. PLoS One 2018; 13:e0208100. [PMID: 30485353 PMCID: PMC6261623 DOI: 10.1371/journal.pone.0208100] [Citation(s) in RCA: 31] [Impact Index Per Article: 4.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/17/2018] [Accepted: 11/12/2018] [Indexed: 12/04/2022] Open
Abstract
Introduction The presence of late gadolinium enhancement (LGE) at the right ventricular insertion point (RVIP) on cardiac magnetic resonance (CMR) is generally believed to be nonspecific, but the clinical implication of this unique LGE pattern in patients with non-ischemic dilated cardiomyopathy (NICM) has not been elucidated. Objectives We investigated the prognostic significance of RVIP-LGE in NICM patients. Methods A total of 360 consecutive NICM patients referred for CMR (102 with no LGE, 50 with RVIP-LGE, 121 with left ventricular [LV]-LGE, and 87 with both an LV and RVIP-LGE) were studied. The primary endpoint was a composite of the all-cause death, hospitalization due to worsening of heart failure, and major arrhythmic events. Results During a mean follow-up of 45.2 ± 36.5 months, 149 (41.4%) patients (22 [21.6%] no LGE vs. 16 [32.0%] RVIP-LGE vs. 62 [51.2%] LV-LGE vs. 49 [56.3%] both LV and RVIP-LGE, P < 0.0001) reached the primary endpoint. A Kaplan Meier curve demonstrated that RVIP-LGE patients had an intermediate trend of an event free survival rate for the composite endpoint (log-rank P < 0.0001). In a multivariable Cox regression model, LV-LGE (P = 0.008) and both LV and RVIP-LGE (P = 0.003) were significantly associated with a worse outcome, whereas RVIP-LGE was not (P = 0.101). In addition, RVIP-LGE patients (n = 32) had a more favorable outcome compared to LV-LGE patients (n = 32) even after matching the extent of the LGE (median 3.4% [interquartile range, 3.1–3.8], 8 [25.0%] RVIP-LGE vs. 20 [62.5%] LV-LGE, P = 0.002). Conclusions LGE confined to the RVIP among NICM patients did not significantly increase the risk of adverse cardiac events, and also showed a better outcome than the same extent of LGE located in the LV. Identification of this unique LGE distribution may help refine the current risk stratification.
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Affiliation(s)
- Jeong-Eun Yi
- Department of Cardiology, Ewha Womans University College of Medicine, Seoul, Republic of Korea
| | - Junbeom Park
- Department of Cardiology, Ewha Womans University College of Medicine, Seoul, Republic of Korea
| | - Hye-Jeong Lee
- Department of Radiology, Research Institute of Radiological Science, Yonsei University College of Medicine, Seoul, Korea
| | - Dong Geum Shin
- Yonsei University Health System, Yonsei Cardiovascular Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Yookyung Kim
- Department of Radiology, College of Medicine, Ewha Womans University School of Medicine, Seoul, Republic of Korea
| | - Minsuk Kim
- Department of Pharmacology, School of Medicine, Ewha Womans University, Seoul, Republic of Korea
| | - Kihwan Kwon
- Department of Cardiology, Ewha Womans University College of Medicine, Seoul, Republic of Korea
| | - Wook Bum Pyun
- Department of Cardiology, Ewha Womans University College of Medicine, Seoul, Republic of Korea
| | - Young Jin Kim
- Department of Radiology, Research Institute of Radiological Science, Yonsei University College of Medicine, Seoul, Korea
- * E-mail: (BYJ); (YJK)
| | - Boyoung Joung
- Yonsei University Health System, Yonsei Cardiovascular Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea
- * E-mail: (BYJ); (YJK)
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2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Heart Rhythm 2018; 15:e73-e189. [DOI: 10.1016/j.hrthm.2017.10.036] [Citation(s) in RCA: 237] [Impact Index Per Article: 33.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/23/2017] [Indexed: 02/07/2023]
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Mirabel M, Damy T, Donal E, Huttin O, Labombarda F, Eicher JC, Cervino C, Laurito M, Offredo L, Tafflet M, Jouven X, Giura G, Desnos M, Jeunemaître X, Empana JP, Charron P, Habib G, Réant P, Hagège A. Influence of centre expertise on the diagnosis and management of hypertrophic cardiomyopathy: A study from the French register of hypertrophic cardiomyopathy (REMY). Int J Cardiol 2018; 275:107-113. [PMID: 30316646 DOI: 10.1016/j.ijcard.2018.09.083] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/04/2018] [Revised: 09/19/2018] [Accepted: 09/24/2018] [Indexed: 11/26/2022]
Abstract
BACKGROUND Our knowledge of hypertrophic cardiomyopathy (HCM) mainly originates from quarternary centres. The objective is to assess the current management of HCM patients in a large multicentre French register according to the level of expertise. METHODS AND RESULTS A total of 1431 HCM patients were recruited across 26 (11 expert and 15 non-expert) centres in REMY, a prospective hospital-based register of adult HCM patients. A sarcomeric origin was suspected in 1284 (89.7%) patients [261 (20.3%) with a reported gene mutation, 242 (18.8%) genotype-negative], while 107 (7.5%) had a diagnosis of non-sarcomeric HCM. Patients managed in non-expert centres were older (P < 0.01) and presented more often with NYHA III/IV class dyspnoea (P < 0.01), congestive heart failure (P < 0.01), low LEVF (P < 0.01), less often with a syncope history (P < 0.01) and lower LV obstruction (P < 0.01) than patients in expert centres. Genotype positive sarcomeric aetiologies were less frequent in non-expert centres (P < 0.01). The use of diagnostic and prognostic tests as cardiac MRI (P < 0.001), genetic (P < 0.001) and alpha-galactosidase A enzyme level testing (P < 0.001), Holter ECG (P < 0.001), and exercise test (P < 0.001), was lower in non-expert centres. Septal ablation procedures using alcohol (P < 0.001) or myectomy (P < 0.001) were more frequent in expert centres. CONCLUSION In real life practice, only a minority of HCM patients are identified as sarcomere positive as per genetic testing. The management of HCM patients varies according to the centre's level of expertise, with less access to diagnostic and prognostic tests in non-expert centres. Non-sarcomeric HCM may therefore be overlooked despite specific treatment in some aetiologies.
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Affiliation(s)
- Mariana Mirabel
- Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Cardiology Department, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France; INSERM CMR970, Paris Cardiovascular Research Center - PARCC, Paris, France
| | - Thibaud Damy
- Assistance Publique-Hôpitaux de Paris, Hôpital Henri Mondor, GRC Amyloid Research Institute, IMRB and Cardiology Department, 94000 Créteil, France
| | - Erwan Donal
- Centre Hospitalo-Universitaire de Rennes, Hôpital Pontchaillou, Cardiology Department,- CIC-IT 1414 and LTSI Inserm U 1099 Université Rennes -1, Rennes, France
| | - Olivier Huttin
- Centre Hospitalo-Universitaire de Nancy, Hôpitaux de Brabois, Cardiology Department, Nancy, France
| | - Fabien Labombarda
- Centre Hospitalo-Universitaire de Caen, Hôpital Côte de Nacre, Cardiology Department, Caen, France
| | - Jean-Christophe Eicher
- Centre Hospitalo-Universitaire de Dijon, Dijon, Hôpital du Bocage, Cardiology Department, France
| | - Claudio Cervino
- Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Cardiology Department, Paris, France
| | - Marianna Laurito
- Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Cardiology Department, Paris, France
| | - Lucile Offredo
- INSERM CMR970, Paris Cardiovascular Research Center - PARCC, Paris, France
| | - Muriel Tafflet
- INSERM CMR970, Paris Cardiovascular Research Center - PARCC, Paris, France
| | - Xavier Jouven
- Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Cardiology Department, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France; INSERM CMR970, Paris Cardiovascular Research Center - PARCC, Paris, France
| | - Geltrude Giura
- Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Cardiology Department, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France
| | - Michel Desnos
- Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Cardiology Department, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France
| | - Xavier Jeunemaître
- Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Department of Genetics, Paris, France
| | | | - Philippe Charron
- Assistance Publique-Hôpitaux de Paris, Hôpital de la Pitié Salpêtrière, Cardiology Department & ICAN, Paris, France
| | - Gilbert Habib
- Assistance Publique Hôpitaux de Marseille, Hôpital La Timone, Cardiology Department, Marseille, France
| | - Patricia Réant
- Centre Hospitalo-Universitaire de Bordeaux, Hôpital Haut Levêque, Cardiology Department, Université de Bordeaux, INSERM 1045, IHU Lyric, Pessac, CIC1401 Bordeaux, France
| | - Albert Hagège
- Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Cardiology Department, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France; INSERM CMR970, Paris Cardiovascular Research Center - PARCC, Paris, France.
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Al-Khatib SM, Stevenson WG, Ackerman MJ, Bryant WJ, Callans DJ, Curtis AB, Deal BJ, Dickfeld T, Field ME, Fonarow GC, Gillis AM, Granger CB, Hammill SC, Hlatky MA, Joglar JA, Kay GN, Matlock DD, Myerburg RJ, Page RL. 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation 2018; 138:e210-e271. [PMID: 29084733 DOI: 10.1161/cir.0000000000000548] [Citation(s) in RCA: 171] [Impact Index Per Article: 24.4] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Affiliation(s)
| | - William G Stevenson
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. Section numbers pertain to those in the full-text guideline. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Michael J Ackerman
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. Section numbers pertain to those in the full-text guideline. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - William J Bryant
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. Section numbers pertain to those in the full-text guideline. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - David J Callans
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. Section numbers pertain to those in the full-text guideline. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Anne B Curtis
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. Section numbers pertain to those in the full-text guideline. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Barbara J Deal
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. Section numbers pertain to those in the full-text guideline. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Timm Dickfeld
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. Section numbers pertain to those in the full-text guideline. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Michael E Field
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. Section numbers pertain to those in the full-text guideline. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Gregg C Fonarow
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. Section numbers pertain to those in the full-text guideline. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Anne M Gillis
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. Section numbers pertain to those in the full-text guideline. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Christopher B Granger
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. Section numbers pertain to those in the full-text guideline. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Stephen C Hammill
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. Section numbers pertain to those in the full-text guideline. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Mark A Hlatky
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. Section numbers pertain to those in the full-text guideline. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - José A Joglar
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. Section numbers pertain to those in the full-text guideline. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - G Neal Kay
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. Section numbers pertain to those in the full-text guideline. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Daniel D Matlock
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. Section numbers pertain to those in the full-text guideline. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Robert J Myerburg
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. Section numbers pertain to those in the full-text guideline. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Richard L Page
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. Section numbers pertain to those in the full-text guideline. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
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49
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Al-Khatib SM, Stevenson WG, Ackerman MJ, Bryant WJ, Callans DJ, Curtis AB, Deal BJ, Dickfeld T, Field ME, Fonarow GC, Gillis AM, Granger CB, Hammill SC, Hlatky MA, Joglar JA, Kay GN, Matlock DD, Myerburg RJ, Page RL. 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation 2018; 138:e272-e391. [PMID: 29084731 DOI: 10.1161/cir.0000000000000549] [Citation(s) in RCA: 294] [Impact Index Per Article: 42.0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Affiliation(s)
| | - William G Stevenson
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Michael J Ackerman
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - William J Bryant
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - David J Callans
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Anne B Curtis
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Barbara J Deal
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Timm Dickfeld
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Michael E Field
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Gregg C Fonarow
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Anne M Gillis
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Christopher B Granger
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Stephen C Hammill
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Mark A Hlatky
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - José A Joglar
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - G Neal Kay
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Daniel D Matlock
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Robert J Myerburg
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
| | - Richard L Page
- Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡HRS Representative. §ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ‖ACC/AHA Task Force on Clinical Practice Guidelines Liaison
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50
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Al-Khatib SM, Stevenson WG, Ackerman MJ, Bryant WJ, Callans DJ, Curtis AB, Deal BJ, Dickfeld T, Field ME, Fonarow GC, Gillis AM, Granger CB, Hammill SC, Hlatky MA, Joglar JA, Kay GN, Matlock DD, Myerburg RJ, Page RL. 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. J Am Coll Cardiol 2018; 72:e91-e220. [PMID: 29097296 DOI: 10.1016/j.jacc.2017.10.054] [Citation(s) in RCA: 800] [Impact Index Per Article: 114.3] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
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