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Mirchuk M. Risk Stratification of Left Ventricle Hypertrabeculation Versus Non-Compaction Cardiomyopathy Using Echocardiography, Magnetic Resonance Imaging, and Cardiac Computed Tomography. Echocardiography 2025; 42:e70165. [PMID: 40309756 DOI: 10.1111/echo.70165] [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: 11/14/2024] [Revised: 03/29/2025] [Accepted: 03/31/2025] [Indexed: 05/02/2025] Open
Abstract
Non-compaction cardiomyopathy (NCCM) is a rare, congenital form of cardiomyopathy characterized by excessive trabeculations in the left ventricle myocardium. NCCM is often an underdiagnosed heart condition characterized by abnormal myocardial trabeculations, which can lead to a wide range of clinical outcomes, from asymptomatic cases to severe heart failure and arrhythmias. It is often diagnosed using imaging techniques like echocardiography, cardiac magnetic resonance imaging (CMR), and cardiac computed tomography (CT), which help assess the heart's structure and function. Although echocardiography remains a key tool, CMR is a gold standard for left ventricle NCCM diagnosis, structural and functional assessment, accurate trabecular quantification, detects fibrosis with late gadolinium enhancement, superior in risk stratification for sudden cardiac death. CT has gained importance in diagnosing NCCM, especially in ruling out coronary artery disease and evaluating complications such as left ventricle thrombus. Accurate risk stratification is crucial for identifying high-risk patients and providing timely interventions. This review examined the contributions of echocardiography, CMR, and cardiac CT in diagnosing NCCM, assessing disease severity, and guiding treatment decisions. We highlighted the strengths and limitations of each imaging modality, discussing their ability to detect myocardial abnormalities, evaluate heart function, and identify fibrosis or other structural changes. We also emphasized integrating imaging findings with clinical and genetic data to enhance patient management and outcomes. Finally, we explored the potential future applications of artificial intelligence in improving diagnostic accuracy and refining risk assessment in NCCM.
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Affiliation(s)
- Marianna Mirchuk
- Department of Radiation Diagnostics, Danylo Halytsky Lviv National Medical University, Lviv, Ukraine
- Ukrainian-Polish Heart Center Lviv, Lviv, Ukraine
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Alfieri M, Principi S, Barbarossa A, Stronati G, Antonicelli R, Casella M, Dello Russo A, Guerra F. How to Approach Left Ventricular Hypertrabeculation: A Practical Guide and Literature Review. J Clin Med 2025; 14:695. [PMID: 39941364 PMCID: PMC11818236 DOI: 10.3390/jcm14030695] [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: 11/30/2024] [Revised: 01/14/2025] [Accepted: 01/19/2025] [Indexed: 02/16/2025] Open
Abstract
Left ventricular hypertrabeculation is one of the most debated conditions in modern cardiology. Many studies have tried to characterise this disease by addressing the various clinical risks and diagnostic tools, but its very nosological existence is currently being challenged. The latest ESC guidelines on cardiomyopathies state that it should be addressed as a morphologic trait rather than an intrinsic disease of the cardiac muscle. Despite the huge number of diagnostic criteria and possible phenocopies, no specific consensus identifies a specific flowchart regarding the management of patients with suspected hypertrabeculation. This review aims to provide a clinical approach for patients with a phenotypical appearance of excessive trabeculation.
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Affiliation(s)
- Michele Alfieri
- Department of Biomedical Sciences and Public Health, Marche Polytechnic University, 60131 Ancona, Italy; (M.A.); (G.S.); (M.C.); (A.D.R.)
- Cardiology Unit IRCCS INRCA, Via della Montagnola 81, 60127 Ancona, Italy;
| | - Samuele Principi
- Cardiology and Arrhythmology Clinic, Department of Cardiovascular Sciences, Marche University Hospital, 60126 Ancona, Italy (A.B.)
| | - Alessandro Barbarossa
- Cardiology and Arrhythmology Clinic, Department of Cardiovascular Sciences, Marche University Hospital, 60126 Ancona, Italy (A.B.)
| | - Giulia Stronati
- Department of Biomedical Sciences and Public Health, Marche Polytechnic University, 60131 Ancona, Italy; (M.A.); (G.S.); (M.C.); (A.D.R.)
- Cardiology Unit IRCCS INRCA, Via della Montagnola 81, 60127 Ancona, Italy;
| | | | - Michela Casella
- Department of Biomedical Sciences and Public Health, Marche Polytechnic University, 60131 Ancona, Italy; (M.A.); (G.S.); (M.C.); (A.D.R.)
- Department of Medical and Surgical Sciences, Marche Polytechnic University, 60131 Ancona, Italy
| | - Antonio Dello Russo
- Department of Biomedical Sciences and Public Health, Marche Polytechnic University, 60131 Ancona, Italy; (M.A.); (G.S.); (M.C.); (A.D.R.)
- Cardiology and Arrhythmology Clinic, Department of Cardiovascular Sciences, Marche University Hospital, 60126 Ancona, Italy (A.B.)
| | - Federico Guerra
- Department of Biomedical Sciences and Public Health, Marche Polytechnic University, 60131 Ancona, Italy; (M.A.); (G.S.); (M.C.); (A.D.R.)
- Cardiology Unit IRCCS INRCA, Via della Montagnola 81, 60127 Ancona, Italy;
- Cardiology and Arrhythmology Clinic, Department of Cardiovascular Sciences, Marche University Hospital, 60126 Ancona, Italy (A.B.)
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Korotkikh A, Vakhnenko Y, Zabolotskikh T, Kazantsev A, Annaev Z. NON-COMPACTION CARDIOMYOPATHY: ISSUES, CONTRADICTIONS AND SEARCH FOR EFFECTIVE DIAGNOSTIC CRITERIA. LITERATURE REVIEW. PART 2. Curr Probl Cardiol 2023; 48:101723. [PMID: 36990189 DOI: 10.1016/j.cpcardiol.2023.101723] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/12/2023] [Revised: 03/22/2023] [Accepted: 03/22/2023] [Indexed: 03/28/2023]
Abstract
Active research of non-compaction cardiomyopathy (NCM) has been going on for more than 30 years. A significant amount of information has been accumulated that is familiar to a much larger number of specialists than in the most recent past. Despite this, numerous issues remain unresolved, ranging from classification (congenital or acquired, nosology or morphological phenotype) to the ongoing search for clear diagnostic criteria that separate NCM from physiological hypertrabecularity and secondary non-compaction myocardium with the background of existing chronic processes. Meanwhile, a high risk of adverse cardiovascular events in a certain group of people with NCM is quite high. These patients need timely and often quite aggressive therapy. This review of sources of scientific and practical information is devoted to the current aspects of the classification, extremely diverse clinical picture, extremely complex genetic and instrumental diagnosis of NCM, and the possibilities of its treatment. The purpose of this review is to analyze current ideas about the controversial problems of non-compaction cardiomyopathy. The material for its preparation is the numerous sources of databases Web Science, PubMed, Google Scholar, eLIBRARY. As a result of their analysis, the authors tried to identify and summarize the main problems of the NCM and identify the ways to resolve them.
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Behind Enemy Lines: Vital Echocardiographic Data Prior to Ventricular Arrhythmia Ablation. Diagnostics (Basel) 2022; 12:diagnostics12092109. [PMID: 36140510 PMCID: PMC9497976 DOI: 10.3390/diagnostics12092109] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/17/2022] [Revised: 08/16/2022] [Accepted: 08/29/2022] [Indexed: 11/17/2022] Open
Abstract
Ventricular arrhythmias (VA) are a major cause of sudden cardiac death (SCD). Echocardiography is the first widely available imaging tool which guides VA management strategies. Along with other invasive and noninvasive imaging techniques, it provides essential information for identification of VA substrate such as differentiation between ischemic and non-ischemic etiology and identification of structural heart disease. Both classic as well as novel echocardiographic techniques such as left ventricular strain measurement and mechanical dispersion assessment provide prognostic information and assist in risk stratification. Furthermore, intracardiac echocardiography may have an adjunctive role for the VA ablation by providing real-time visualization of cardiac structures, continuous monitoring of catheter location and early recognition of procedural complications. This review gathers all relevant information that echocardiography may offer prior to VA ablation procedures.
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Hotta VT, Martinelli LMB, Moisés VA, Mady C, Fernandes F. Left ventricular noncompaction highlighted by three-dimensional and speckle tracking echocardiography. EINSTEIN-SAO PAULO 2022; 20:eAI6853. [PMID: 35976353 PMCID: PMC9377308 DOI: 10.31744/einstein_journal/2022ai6853] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/17/2021] [Accepted: 01/06/2022] [Indexed: 11/11/2022] Open
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Left Ventricular Non-Compaction Cardiomyopathy-Still More Questions than Answers. J Clin Med 2022; 11:jcm11144135. [PMID: 35887898 PMCID: PMC9315982 DOI: 10.3390/jcm11144135] [Citation(s) in RCA: 14] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/28/2022] [Revised: 07/12/2022] [Accepted: 07/13/2022] [Indexed: 12/24/2022] Open
Abstract
Left ventricular non-compaction (LVNC) describes the phenotypical phenomena characterized by the presence of excessive trabeculation of the left ventricle which forms a deep recess filled with blood. Considering the lack of a uniform definition of LVNC as well as the "golden standard" it is difficult to estimate the actual incidence of the disease, however, seems to be overdiagnosed, due to unspecific diagnostic criteria. The non-compacted myocardium may appear both as a disease representation or variant of the norm or as an adaptive phenomenon. This article covers different approaches to incidence, pathogenesis, diagnostics, and treatment of LVNC as well as recommendations for patients during follow-up.
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Yildirim G, Dursun M, Arslan R. Effect of trabeculated myocardial mass on left ventricle global and regional functions in noncompaction cardiomyopathy. World J Cardiol 2021; 13:211-222. [PMID: 34367505 PMCID: PMC8326155 DOI: 10.4330/wjc.v13.i7.211] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/15/2020] [Revised: 04/12/2021] [Accepted: 04/29/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Left ventricular (LV) noncompaction cardiomyopathy is a rare cardiomyopathic subtype that has been recognized in recent years and is being diagnosed at an increased rate. There is no consensus regarding the diagnosis of the disease, and increased trabeculation rates that meet the existing diagnostic criteria may even be present in healthy asymptomatic people. This indicates that differentiating criteria for diagnosis are needed.
AIM To examine the increase in myocardial trabeculation and the change in left ventricular global and regional functions.
METHODS This retrospective study included 65 patients (28 females, 37 males) diagnosed with LV noncompaction cardiomyopathy who underwent cardiac magnetic resonance imaging between January 2011 and August 2016 and had a noncompacted/compacted myocardial thickness ratio of over 2.3 in more than one segment in the left ventricle. The distribution and ratios of trabeculations in apical, midventricular, and basal regions were examined in short-axis images obtained from cardiac magnetic resonance. In addition, by using short-axis cine images, regional ejection fraction (EF) and global EF were calculated using the Simpson method in the left ventricle at apical, basal, and midventricular levels.
RESULTS While the number of trabeculated segments were similar at the apical (3.2 ± 1.0) and midventricular levels, a statistically significant level of involvement was not observed at the basal level (0.4 ± 0.9) (P > 0.05). The highest noncompacted/compacted (trabeculation) ratio was observed at the apical level (3.9 ± 1.4), while this ratio was higher at the anterior (59%-89.4%) and lateral (62%-84.8%) segments (P > 0.05). Global EF was positively correlated with apical, midventricular, and basal regional EF (P < 0.05). However, there was no significant correlation between regional EF and the number of trabeculated segments or trabeculation ratio in all three regions; nor was there a significant correlation between regional EF and the number of trabeculated segments or trabeculation ratio in the entire LV (P > 0.05).
CONCLUSION No global or regional relationship was observed between LV dysfunction and trabeculation rate or the number of trabeculated segments. This limits the usefulness of change in LV functions in the differentiation between normal and pathological trabeculation.
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Affiliation(s)
- Gulsah Yildirim
- Department of Radiology, University of Health Sciences, Istanbul Fatih Sultan Mehmet Training and Research Hospital, İstanbul 34752, Turkey
| | - Memduh Dursun
- Department of Radiology, İstanbul University School of Medicine, İstanbul 34752, Turkey
| | - Rıfat Arslan
- Department of Radiology, Istanbul University, Istanbul Faculty of Medicine, İstanbul 34752, Turkey
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Srivastava S, Yavari M, Al-Abcha A, Banga S, Abela G. Ventricular non-compaction review. Heart Fail Rev 2021; 27:1063-1076. [PMID: 34232438 DOI: 10.1007/s10741-021-10128-3] [Citation(s) in RCA: 23] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 06/09/2021] [Indexed: 11/24/2022]
Abstract
Left ventricular non-compaction cardiomyopathy (LVNC) is a rare and unclassified cardiomyopathy that carries the potential to cause heart failure, arrhythmias, and embolic events within adults. The diagnosis of this cardiomyopathy can be based off a variety of echocardiographic, cardiac magnetic resonance (CMR), and computed tomography (CT) imaging criteria; none of which have been standardized to establish a firm diagnosis. This is further complicated by the observation from prior studies that LVNC may present as different forms of cardiomyopathy, each with its own subset of nuances that may change treatment strategies. Management of such cardiomyopathy has been debated in terms of anticoagulation, electrophysiologic studies to prevent arrhythmia, as well as heart failure prevention. Not enough data exists in regard to establishing firm guidelines for management. The following article aims to provide a comprehensive review in regard to the etiologies, pathogenesis, diagnostic criteria, management, and treatment of LVNC.
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Affiliation(s)
- Shaurya Srivastava
- Department of Internal Medicine, Michigan State University, East Lansing, USA.
| | - Majid Yavari
- Department of Internal Medicine, Michigan State University, East Lansing, USA
| | - Abdullah Al-Abcha
- Department of Internal Medicine, Michigan State University, East Lansing, USA
| | - Sandeep Banga
- Sparrow Hospital, Transthoracic Cardiovascular Institute, Lansing, USA
| | - George Abela
- Department of Internal Medicine, Michigan State University, East Lansing, USA
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Markousis-Mavrogenis G, Giannakopoulou A, Andreou N, Papadopoulos G, Vartela V, Kolovou G, Bacopoulou F, Tsarouhas K, Kanaka-Gantenbein C, A Spandidos D, I Mavrogeni S. Cardiovascular magnetic resonance clarifies arrhythmogenicity in asymptomatic young athletes with ventricular arrhythmias undergoing pre-participation evaluation. Exp Ther Med 2020; 20:561-571. [PMID: 32537014 PMCID: PMC7282016 DOI: 10.3892/etm.2020.8693] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/02/2020] [Accepted: 04/29/2020] [Indexed: 12/14/2022] Open
Abstract
Pre-participation sports examination (PPE) is a frequent reason for consultation. However, the exact role of cardiovascular magnetic resonance (CMR) in PPE remains undefined. The additive value of CMR in adolescent athletes with ventricular rhythm disturbances (VRDs) was investigated. We prospectively recruited and evaluated with CMR 50 consecutive, asymptomatic young athletes referred to our tertiary center after identification of VRDs on electrocardiogram (ECG) with otherwise normal standard PPE and echocardiography, and 20 age- and sex-matched healthy volunteer athletes who underwent the same evaluations. The primary outcome was case-control status and the secondary outcome was the discrimination between athletes with VRDs with and without non-sustained ventricular tachycardia (VT). CMR identified arrhythmogenic substrates in all athletes with VRDs. The predominant condition was myocarditis and arrhythmogenic right ventricular cardiomyopathy in patients with and without VT, respectively. Based on penalized regression analysis, late gadolinium enhancement (LGE), early gadolinium enhancement (EGE), extracellular volume fraction (ECV), and T2-mapping, best distinguished between case-control status. The aforementioned indices predicted case-control status independent of age and sex: EGE [Odds ratio (95% confidence interval): 6.89 (2.19-21.62) per 0.5-unit, P<0.001], LGE (perfect prediction), ECV [1.66 (1.25-2.22), P<0.001] and T2 mapping [1.40 (1.13-1.72), P=0.002], among other independent CMR-derived predictors. Only indexed ventricular volumes independently discriminated between VRD patients with and without VT. In this study, asymptomatic young athletes with VRDs and normal PPE/echocardiography were optimally discriminated from healthy control athletes by CMR-derived indices, and CMR allowed for the identification of arrhythmogenic substrates in all cases.
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Affiliation(s)
| | | | - Nikolaos Andreou
- Department of Cardiology, Aghia Sophia Children's Hospital, Athens 11527, Greece
| | - George Papadopoulos
- Department of Cardiology, Aghia Sophia Children's Hospital, Athens 11527, Greece
| | | | | | - Flora Bacopoulou
- Exercise Physiology and Sports Medicine Clinic, Center for Adolescent Medicine and UNESCO Chair on Adolescent Health Care, First Department of Pediatrics, Medical School, National and Kapodistrian University of Athens, Aghia Sophia Children's Hospital, Athens 11527, Greece
| | - Konstantinos Tsarouhas
- Exercise Physiology and Sports Medicine Clinic, Center for Adolescent Medicine and UNESCO Chair on Adolescent Health Care, First Department of Pediatrics, Medical School, National and Kapodistrian University of Athens, Aghia Sophia Children's Hospital, Athens 11527, Greece.,Department of Cardiology, University Hospital of Larissa, Larissa 41334, Greece
| | - Christina Kanaka-Gantenbein
- Exercise Physiology and Sports Medicine Clinic, Center for Adolescent Medicine and UNESCO Chair on Adolescent Health Care, First Department of Pediatrics, Medical School, National and Kapodistrian University of Athens, Aghia Sophia Children's Hospital, Athens 11527, Greece
| | - Demetrios A Spandidos
- Laboratory of Clinical Virology, School of Medicine, University of Crete, Heraklion 71003, Greece
| | - Sophie I Mavrogeni
- Onassis Cardiac Surgery Center, Athens 17674, Greece.,Exercise Physiology and Sports Medicine Clinic, Center for Adolescent Medicine and UNESCO Chair on Adolescent Health Care, First Department of Pediatrics, Medical School, National and Kapodistrian University of Athens, Aghia Sophia Children's Hospital, Athens 11527, Greece
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