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Lauriero F, Vita CV, Perazzolo A, Sanseverino G, Moliterno E, Rovere G, Marano R, Natale L. Acute Myocarditis and Inflammatory Cardiomyopathies: Insights From Cardiac Magnetic Resonance Findings. Echocardiography 2025; 42:e70099. [PMID: 39963997 PMCID: PMC11834149 DOI: 10.1111/echo.70099] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/05/2024] [Revised: 01/12/2025] [Accepted: 01/29/2025] [Indexed: 02/21/2025] Open
Abstract
Myocardial inflammation encompasses a broad spectrum of conditions, including acute myocarditis, chronic inflammatory cardiomyopathy, and several overlapping entities that differ in clinical presentation, pathophysiology, and progression. These conditions range from self-limiting acute inflammation to chronic myocardial injury and dysfunction. The etiologic classification of myocardial inflammation highlights the complexity of its pathogenesis, involving direct tissue damage, immune-mediated mechanisms, and environmental triggers. Cardiac magnetic resonance (CMR) imaging has become a central diagnostic tool in the assessment of myocardial inflammation, providing precise characterization of myocardial tissue, assessing cardiac function, and stratifying prognosis. Advanced techniques such as T1 and T2 mapping and extracellular volume quantification have further expanded its diagnostic capabilities. This review highlights the essential role of CMR in diagnosing myocardial inflammation, recognizing various imaging findings associated with different underlying causes, and informing clinical management. The standardization of CMR protocols, along with advancements in imaging techniques and strengthened interdisciplinary collaboration, represents a fundamental step toward improving diagnostic accuracy, patient outcomes, and the understanding of the broad spectrum of myocardial inflammatory diseases.
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Affiliation(s)
- Francesco Lauriero
- Department of Diagnostic Imaging, Oncological Radiotherapy and HematologyFondazione Policlinico Universitario Agostino Gemelli IRCCSRomeItaly
| | - Camilla Vittoria Vita
- Department of Radiological and Haematological Sciences–Section of RadiologyUniversità Cattolica del Sacro CuoreRomeItaly
| | - Alessio Perazzolo
- Department of Radiological and Haematological Sciences–Section of RadiologyUniversità Cattolica del Sacro CuoreRomeItaly
| | - Giovanni Sanseverino
- Department of Radiological and Haematological Sciences–Section of RadiologyUniversità Cattolica del Sacro CuoreRomeItaly
| | - Eleonora Moliterno
- Department of Radiological and Haematological Sciences–Section of RadiologyUniversità Cattolica del Sacro CuoreRomeItaly
| | - Giuseppe Rovere
- Department of Diagnostic Imaging, Oncological Radiotherapy and HematologyFondazione Policlinico Universitario Agostino Gemelli IRCCSRomeItaly
| | - Riccardo Marano
- Department of Diagnostic Imaging, Oncological Radiotherapy and HematologyFondazione Policlinico Universitario Agostino Gemelli IRCCSRomeItaly
- Department of Radiological and Haematological Sciences–Section of RadiologyUniversità Cattolica del Sacro CuoreRomeItaly
| | - Luigi Natale
- Department of Diagnostic Imaging, Oncological Radiotherapy and HematologyFondazione Policlinico Universitario Agostino Gemelli IRCCSRomeItaly
- Department of Radiological and Haematological Sciences–Section of RadiologyUniversità Cattolica del Sacro CuoreRomeItaly
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Fairley JL, Ross L, Quinlivan A, Hansen D, Paratz E, Stevens W, Kistler PM, McLellan A, La Gerche A, Nikpour M. Sudden cardiac death, arrhythmias and abnormal electrocardiography in systemic sclerosis: A systematic review and meta-analysis. Semin Arthritis Rheum 2023; 62:152229. [PMID: 37354723 DOI: 10.1016/j.semarthrit.2023.152229] [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: 03/03/2023] [Revised: 05/15/2023] [Accepted: 05/17/2023] [Indexed: 06/26/2023]
Abstract
OBJECTIVE To calculate the frequency of sudden cardiac death(SCD), arrhythmia and conduction defects in SSc. METHODS MEDLINE/EMBASE were searched to January 2023. English-language studies reporting the incidence/frequency of SCD, arrhythmia and electrocardiography(ECG) abnormalities in SSc were included. Odds ratios(OR), estimations of annual incidence or pooled frequencies were calculated. RESULTS Seventy-nine studies(n = 13,609 participants with SSc) were included in the meta-analysis. Methodology and outcomes were heterogeneous. Ten studies included cohorts with known/suspected SSc-associated heart involvement(SHI), generally defined as clinically-manifest cardiac disease/abnormal cardiac investigations. The incidence of SCD in SHI was estimated to be 3.3% annually(n = 4 studies, 301PY follow-up). On ambulatory ECG, 18% of SHI cohorts had non-sustained ventricular tachycardia(NSVT; n = 4, 95%CI3.2-39.3%), 70% frequent premature ventricular complexes (PVCs; n = 1, 95%CI34.8-93.3%), and 8% atrial fibrillation (AF; n = 1, 95%CI4.2-13.6%). Nineteen studies included participants without SHI, defined as normal cardiac investigations/absence of cardiac disease. The estimated incidence of SCD was approximately 2.9% annually (n = 1, 67.5PY). Compared to healthy controls, individuals without SHI demonstrated NSVT 13.3-times more frequently (n = 2, 95%CI2-102), and paroxysmal supraventricular tachycardia 7-times more frequently (n = 4, 95%CI3-15). Other ambulatory ECG abnormalities included NSVT in 9% (n = 7, 95%CI6-14%), >1000 PVCs/24 h in 6% (n = 2, 95%CI1-13%), and AF in 7% (n = 5, 0-21%). Fifty studies included general SSc cohorts unselected for cardiac disease. The incidence of SCD was estimated to be 2.0% annually(n = 4 studies, 1646PY). Unselected SSc cohorts were 10.5-times more likely to demonstrate frequent PVCs (n = 2, 95%CI 2-59) and 2.5-times more likely to have an abnormal electrocardiography (n = 2, 95%CI1-4). CONCLUSIONS The incidence of SCD in SSc is estimated to be 1.0-3.3% annually, at least 10-fold higher than general population estimates. Arrhythmias including NSVT and frequent PVCs appear common, including amongst those without known/suspected SHI.
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Affiliation(s)
- Jessica L Fairley
- The University of Melbourne, Melbourne, Victoria, Australia; St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia
| | - Laura Ross
- The University of Melbourne, Melbourne, Victoria, Australia; St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia
| | - Alannah Quinlivan
- The University of Melbourne, Melbourne, Victoria, Australia; St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia
| | - Dylan Hansen
- St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia
| | - Elizabeth Paratz
- St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia; The Baker Heart and Diabetes Institute, Melbourne, Victoria, Australia
| | - Wendy Stevens
- The University of Melbourne, Melbourne, Victoria, Australia; St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia
| | - Peter M Kistler
- The University of Melbourne, Melbourne, Victoria, Australia; The Baker Heart and Diabetes Institute, Melbourne, Victoria, Australia; The Alfred Hospital, Melbourne, Victoria, Australia; Monash University, Melbourne, Australia
| | - Alex McLellan
- St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia
| | - Andre La Gerche
- The University of Melbourne, Melbourne, Victoria, Australia; St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia; The Baker Heart and Diabetes Institute, Melbourne, Victoria, Australia
| | - Mandana Nikpour
- The University of Melbourne, Melbourne, Victoria, Australia; St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia.
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Gulhane A, Ordovas K. Cardiac magnetic resonance assessment of cardiac involvement in autoimmune diseases. Front Cardiovasc Med 2023; 10:1215907. [PMID: 37808881 PMCID: PMC10556673 DOI: 10.3389/fcvm.2023.1215907] [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: 05/02/2023] [Accepted: 09/11/2023] [Indexed: 10/10/2023] Open
Abstract
Cardiac magnetic resonance (CMR) is emerging as the modality of choice to assess early cardiovascular involvement in patients with autoimmune rheumatic diseases (ARDs) that often has a silent presentation and may lead to changes in management. Besides being reproducible and accurate for functional and volumetric assessment, the strength of CMR is its unique ability to perform myocardial tissue characterization that allows the identification of inflammation, edema, and fibrosis. Several CMR biomarkers may provide prognostic information on the severity and progression of cardiovascular involvement in patients with ARDs. In addition, CMR may add value in assessing treatment response and identification of cardiotoxicity related to therapy with immunomodulators that are commonly used to treat these conditions. In this review, we aim to discuss the following objectives: •Illustrate imaging findings of multi-parametric CMR approach in the diagnosis of cardiovascular involvement in various ARDs;•Review the CMR signatures for risk stratification, prognostication, and guiding treatment strategies in ARDs;•Describe the utility of routine and advanced CMR sequences in identifying cardiotoxicity related to immunomodulators and disease-modifying agents in ARDs;•Discuss the limitations of CMR, recent advances, current research gaps, and potential future developments in the field.
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Affiliation(s)
- Avanti Gulhane
- Department of Radiology, University of Washington, School of Medicine, Seattle, WA, United States
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Pussadhamma B, Mahakkanukrauh A, Suwannaroj S, Nanagara R, Foocharoen C. Clinical outcomes of asymptomatic cardiac involvement in systemic sclerosis patients after a 2-year follow-up (extended study). Am J Med Sci 2021; 362:570-577. [PMID: 34087232 DOI: 10.1016/j.amjms.2021.05.027] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/03/2020] [Revised: 05/07/2021] [Accepted: 05/28/2021] [Indexed: 11/19/2022]
Abstract
BACKGROUND Asymptomatic cardiac involvement in systemic sclerosis (SSc) has been reported. Long-term follow-up might elucidate the clinical implications of these abnormalities. The aim was to identify the clinical outcomes of asymptomatic cardiac involvement in SSc patients after 2 years of follow-up. METHODS A cohort study was done at Khon Kaen University, Thailand, on adult patients with SSc who completed the preliminary study. Repeated investigations included electrocardiography, chest radiography, echocardiography, and blood tests for creatine kinase-MB, high sensitivity cardiac troponin-T, and N-terminal prohormone of brain natriuretic peptide. RESULTS Seventy-four of the 103 patients from the previous study were enrolled. The mean duration of follow-up was 3.1±0.9 years. Five patients developed symptomatic cardiac involvement-all of whom had pulmonary arterial hypertension (PAH). The incidence of symptomatic cardiac involvement for the combined 315 person-years was 1.6 per 100-person-years (95%CI 0.7-3.4). Fourteen patients died resulting in a mortality incidence of 4.4 per 100-person-years (95%CI 4.3-5.4). Persistent cardiac involvement was found in 35 patients for an incidence of 11.1 per 100-person-years (95%CI 8.0-15.5). Two of the patients who had persistent elevated cardiac enzyme developed PAH at a respective 3.7 and 39.4 months after the initial evaluation. None of the clinical parameters were predictive of symptomatic and persistent cardiac involvement. Only male sex was associated with mortality (hazard ratio 3.70; 95%CI 1.22-11.11). CONCLUSIONS Cardiac involvement in SSc can progress slowly or even be reversed. Based on a previous test, the incidence of symptomatic cardiac involvement after 2 years was low despite its being a persistent involvement. If symptomatic cardiac involvement develops, PAH is the most prevalent symptom.
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Affiliation(s)
- Burabha Pussadhamma
- Division of Cardiology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand
| | - Ajanee Mahakkanukrauh
- Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand
| | - Siraphop Suwannaroj
- Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand
| | - Ratanavadee Nanagara
- Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand
| | - Chingching Foocharoen
- Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.
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Colaci M, Dal Bosco Y, Schinocca C, Ronsivalle G, Guggino G, De Andres I, Russo AA, Sambataro D, Sambataro G, Malatino L. Aortic root dilation in associated with the reduction in capillary density observed at nailfold capillaroscopy in SSc patients. Clin Rheumatol 2020; 40:1185-1189. [PMID: 32506312 DOI: 10.1007/s10067-020-05201-5] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/18/2020] [Revised: 05/15/2020] [Accepted: 05/22/2020] [Indexed: 01/08/2023]
Abstract
Systemic sclerosis (SSc) is an autoimmune disease characterized by endothelial dysfunction and fibroblasts activation. Microvascular disease may be easily observed by means of nailfold capillaroscopy. Recent evidences emphasized also the involvement of large-medium arteries in SSc, mainly in terms of increased stiffness of the vessel wall. The study aims to measure aortic root diameter in a cohort of SSc patients and to correlate echocardiographic findings with the capillaroscopic pictures. We analyzed the clinical records of 125 consecutive SSc patients (M/F 14/111, mean age 55 ± 12.7 years, median disease duration 11 years) referring in 3 second-level rheumatology centers. All subjects underwent to heart ultrasound evaluation and videocapillaroscopic evaluation. At capillaroscopy, the patients with early SSc pattern belonged to the subgroup 1, while those with the active/late patterns (characterized by the reduction of capillary density) belonged to the subgroup 2. We found aortic root dilation in 8 (6.4%) SSc patients, with a mean value of 37.8 ± 1.2 mm (range 37-40 mm). Aortic root dilation was observed in only one patient in the subgroup 1 (1/62, 1.6%) and in 7 cases of the subgroup 2 (7/63, 11.1%; p = 0.03). Our study found a significant association between aortic root dilation and impairment of capillary density at nailfold videocapillaroscopy in SSc patients. We hypothesize that SSc-related microangiopathy revealed by nailfold videocapillaroscopy could mimic that of aortic vasa vasorum, contributing to deteriorate the aortic wall structure and favoring aortic root dilation and stiffening.
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Affiliation(s)
- Michele Colaci
- Rheumatology Centre, Internal Medicine Unit, Department of Clinical and Experimental Medicine, c/o Azienda Ospedaliera per l'Emergenza Cannizzaro, University of Catania, Catania, Italy.
| | - Ylenia Dal Bosco
- Rheumatology Centre, Internal Medicine Unit, Department of Clinical and Experimental Medicine, c/o Azienda Ospedaliera per l'Emergenza Cannizzaro, University of Catania, Catania, Italy
| | - Claudia Schinocca
- Rheumatology Centre, Internal Medicine Unit, Department of Clinical and Experimental Medicine, c/o Azienda Ospedaliera per l'Emergenza Cannizzaro, University of Catania, Catania, Italy
| | | | - Giuliana Guggino
- Rheumatology Unit, Policlinico "P. Giaccone", Università di Palermo, Palermo, Italy
| | - Ilenia De Andres
- Rheumatology Unit, Azienda Ospedaliera di Rilievo Nazionale ed Alta Specializzazione "Garibaldi", Catania, Italy
| | - Alessandra A Russo
- Rheumatology Unit, Azienda Ospedaliera di Rilievo Nazionale ed Alta Specializzazione "Garibaldi", Catania, Italy
| | - Domenico Sambataro
- Rheumatology Centre, Internal Medicine Unit, Department of Clinical and Experimental Medicine, c/o Azienda Ospedaliera per l'Emergenza Cannizzaro, University of Catania, Catania, Italy
| | - Gianluca Sambataro
- Artroreuma srl, Outpatient Clinic of Rheumatology associated with the National Health System, Corso S. Vito 53, 95030, Catania, Italy
| | - Lorenzo Malatino
- Internal Medicine Unit, Azienda Ospedaliera per l'Emergenza Cannizzaro, University of Catania, Catania, Italy
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Ross L, Prior D, Proudman S, Vacca A, Baron M, Nikpour M. Defining primary systemic sclerosis heart involvement: A scoping literature review. Semin Arthritis Rheum 2019; 48:874-887. [PMID: 30170705 DOI: 10.1016/j.semarthrit.2018.07.008] [Citation(s) in RCA: 39] [Impact Index Per Article: 6.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/15/2018] [Revised: 07/09/2018] [Accepted: 07/20/2018] [Indexed: 12/20/2022]
Abstract
BACKGROUND Clinically evident primary heart involvement due to systemic sclerosis (SHI) is considered a poor prognostic factor and is a leading cause of systemic sclerosis (SSc) related death. Yet, there remains no consensus definition of SHI and poor understanding of the natural history and risk factors for the development of SHI. METHODS We performed a scoping literature review of published articles with a primary focus of SHI to capture previously used definitions of SHI and items used to measure SHI. Any factors reported to be associated with an increased risk of SHI were recorded. RESULTS Of the 2436 records identified in a search of MEDLINE, EMBASE and PubMed databases, 295 were included in the final scoping review. Analysis of the literature revealed studies of variable quality, generally low patient numbers and highly heterogeneous definitions of SHI within studies. There is no clear consensus from the literature as to the scope of SHI and the prognostic significance of sub-clinical investigation abnormalities commonly detected. CONCLUSION The lack of a standardised definition of SHI remains a significant unmet need in SSc. The results of this review will assist in the development of consensus classification criteria to enable more accurate quantification of the burden of SHI, identification of factors associated with increased risk of developing SHI, and evaluation of the efficacy of any novel therapeutic strategies.
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Affiliation(s)
- Laura Ross
- Department of Medicine at St Vincent's Hospital, University of Melbourne, 41 Victoria Parade, Fitzroy, VIC 3065, Australia; Department of Rheumatology, St Vincent's Hospital, 41 Victoria Parade, Fitzroy, VIC 3065, Australia.
| | - David Prior
- Department of Medicine at St Vincent's Hospital, University of Melbourne, 41 Victoria Parade, Fitzroy, VIC 3065, Australia; Department of Cardiology, St Vincent's Hospital, 41 Victoria Parade, Fitzroy, VIC 3065, Australia.
| | - Susanna Proudman
- Rheumatology Unit, Royal Adelaide Hospital, Port Road, Adelaide, SA 5000, Australia; Discipline of Medicine, University of Adelaide, Adelaide, SA 5000, Australia.
| | - Alessandra Vacca
- Unit of Rheumatology, University Hospital of Cagliari, S.S. 554, bivio per Sestu, 09042 Monserrato (CA), Italy..
| | - Murray Baron
- Division of Rheumatology, Jewish General Hospital, McGill University, 5750 Côtes-des-Neiges Rd, Montreal, QC H3S 1Y9, Canada.
| | - Mandana Nikpour
- Department of Medicine at St Vincent's Hospital, University of Melbourne, 41 Victoria Parade, Fitzroy, VIC 3065, Australia; Department of Rheumatology, St Vincent's Hospital, 41 Victoria Parade, Fitzroy, VIC 3065, Australia.
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Fernández Morales A, Iniesta N, Fernández-Codina A, Vaz de Cunha J, Pérez Romero T, Hurtado García R, Simeón-Aznar CP, Fonollosa V, Cervera R, Espinosa G. Cardiac tamponade and severe pericardial effusion in systemic sclerosis: report of nine patients and review of the literature. Int J Rheum Dis 2016; 20:1582-1592. [PMID: 27943614 DOI: 10.1111/1756-185x.12952] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/03/2023]
Abstract
AIM To describe the clinical characteristics, treatment and outcome of patients with systemic sclerosis (SSc) developing severe pericardial effusion or cardiac tamponade. METHODS SSc patients with severe pericardial effusion or cardiac tamponade from three Spanish hospitals were collected. In addition, a computer-assisted (PubMed, MEDLINE) search of the literature to identify all cases of cardiac tamponade or severe pericardial effusion associated with SSc reported in English, French and Spanish from 1987 through September 2015 was performed. RESULTS We included 40 patients (nine cases from the Spanish hospitals and 31 cases from the literature review). Most patients (87%) were female with a mean age at pericardial involvement of 49.3 ± 15.2 years (range: 18-80 years), and 22 (55%) patients had the diffuse cutaneous subtype. Twenty-five (63%) patients presented with cardiac tamponade and the remaining 15 (37%) as severe or massive pericardial effusion. Pericardial involvement was previous or simultaneous to SSc diagnosis in 13 (32.5%) cases. In most cases (88.9%) pericardial fluid analysis disclosed an exudate. Half the patients received steroids and 35% needed surgical treatment. Five (12.5%) patients died due to cardiac tamponade, three of them during the acute phase and the remaining two, 2 and 9 months later, respectively. CONCLUSIONS Although cardiac tamponade or severe pericardial effusion is an infrequent complication in SSc patients, it can be the first manifestation of disease associated with the diffuse cutaneous subset. No specific treatment for this complication is known.
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Affiliation(s)
| | - Nerea Iniesta
- Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain
| | | | | | | | | | | | - Vicent Fonollosa
- Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Catalonia, Spain
| | - Ricard Cervera
- Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain
| | - Gerard Espinosa
- Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain
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Foocharoen C, Pussadhamma B, Mahakkanukrauh A, Suwannaroj S, Nanagara R. Asymptomatic cardiac involvement in Thai systemic sclerosis: prevalence and clinical correlations with non-cardiac manifestations (preliminary report). Rheumatology (Oxford) 2015; 54:1616-21. [DOI: 10.1093/rheumatology/kev096] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2014] [Indexed: 11/13/2022] Open
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Lambova S. Cardiac manifestations in systemic sclerosis. World J Cardiol 2014; 6:993-1005. [PMID: 25276300 PMCID: PMC4176808 DOI: 10.4330/wjc.v6.i9.993] [Citation(s) in RCA: 114] [Impact Index Per Article: 10.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/29/2013] [Revised: 05/11/2014] [Accepted: 07/15/2014] [Indexed: 02/06/2023] Open
Abstract
Primary cardiac involvement, which develops as a direct consequence of systemic sclerosis (SSc), may manifest as myocardial damage, fibrosis of the conduction system, pericardial and, less frequently, as valvular disease. In addition, cardiac complications in SSc may develop as a secondary phenomenon due to pulmonary arterial hypertension and kidney pathology. The prevalence of primary cardiac involvement in SSc is variable and difficult to determine because of the diversity of cardiac manifestations, the presence of subclinical periods, the type of diagnostic tools applied, and the diversity of patient populations. When clinically manifested, cardiac involvement is thought to be an important prognostic factor. Profound microvascular disease is a pathognomonic feature of SSc, as both vasospasm and structural alterations are present. Such alterations are thought to predict macrovascular atherosclerosis over time. There are contradictory reports regarding the prevalence of atherosclerosis in SSc. According to some authors, the prevalence of atherosclerosis of the large epicardial coronary arteries is similar to that of the general population, in contrast with other rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus. However, the level of inflammation in SSc is inferior. Thus, the atherosclerotic process may not be as aggressive and not easily detectable in smaller studies. Echocardiography (especially tissue Doppler imaging), single-photon emission computed tomography, magnetic resonance imaging and cardiac computed tomography are sensitive techniques for earlier detection of both structural and functional scleroderma-related cardiac pathologies. Screening for subclinical cardiac involvement via modern, sensitive tools provides an opportunity for early diagnosis and treatment, which is of crucial importance for a positive outcome.
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Someya F, Mugii N. Pulmonary rehabilitation outcome of exercise-induced oxygen desaturation in systemic sclerosis with interstitial lung disease. Health (London) 2013. [DOI: 10.4236/health.2013.56a2001] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
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Allanore Y, Wahbi K, Borderie D, Weber S, Kahan A, Meune C. N-terminal pro-brain natriuretic peptide in systemic sclerosis: a new cornerstone of cardiovascular assessment? Ann Rheum Dis 2009; 68:1885-9. [PMID: 19054819 DOI: 10.1136/ard.2008.098087] [Citation(s) in RCA: 37] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
Abstract
BACKGROUND Cardiac involvement, a common and often fatal complication of systemic sclerosis (SSc), is currently detected by standard echocardiography enhanced by tissue Doppler echocardiography (TDE). OBJECTIVE The performance of the biomarker of cardiovascular disease, N-terminal pro-brain natriuretic peptide (NT-proBNP), in the detection of cardiac involvement by SSc was examined. METHODS A total of 69 consecutive patients with SSc (mean (SD) age 56 (13) years, 56 women) were prospectively studied with standard echocardiography and TDE measurements of longitudinal mitral and tricuspid annular velocities. Plasma NT-proBNP was measured in all patients. RESULTS Overall, 18 patients had manifestations of cardiac involvement, of whom 7 had depressed left ventricular and 8 depressed right ventricular myocardial contractility, and 8 had elevated systolic pulmonary arterial pressure. Patients with reduced contractility had increased mean (SD) NT-proBNP (704 (878) pg/ml versus 118 (112) pg/ml in patients with normal myocardial contractility, p<0.001). Similarly, NT-proBNP was higher in patients with (607 (758) pg/ml) than in patients without (96 (78) pg/ml) manifestations of overall cardiac involvement (p<0.001). Receiver operating characteristic analysis showed NT-proBNP reliably detected depressed myocardial contractility and overall cardiac involvement (area under the curve 0.905 (95% CI 0.814 to 0.996) and 0.935 (95% CI 0.871 to 0.996), respectively). Considering patients with SSc with normal echocardiography and TDE as controls, and using a 125 pg/ml cut-off concentration, sensitivity and specificity were 92% and 71% in the detection of depressed myocardial contractility, and 94% and 78% for overall cardiac involvement. CONCLUSIONS NT-proBNP reliably detected the presence of cardiac involvement and appears to be a very useful marker to risk stratify patients presenting with SSc.
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Affiliation(s)
- Y Allanore
- Department of Rheumatology A, Paris Descartes University, Cochin Hospital, APHP, 75014 Paris, France.
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Meune C, Vignaux O, Kahan A, Allanore Y. Heart involvement in systemic sclerosis: evolving concept and diagnostic methodologies. Arch Cardiovasc Dis 2009; 103:46-52. [PMID: 20142120 DOI: 10.1016/j.acvd.2009.06.009] [Citation(s) in RCA: 97] [Impact Index Per Article: 6.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/05/2009] [Revised: 06/30/2009] [Accepted: 06/30/2009] [Indexed: 12/16/2022]
Abstract
Heart involvement, including primary myocardial involvement, is very common in systemic sclerosis. There is strong evidence that primary myocardial involvement is related to repeat focal ischaemic injury causing subsequent irreversible myocardial fibrosis. Clinically evident cardiac involvement is recognized to be a poor prognostic factor; thus preclinical identification is highly encouraged. The severity of heart involvement has been confirmed recently. Echocardiography, including pulsed tissue Doppler echocardiography, is the cornerstone of routine heart assessment. Myocardial perfusion may be assessed by single photon emission computed tomography. If available, cardiac magnetic resonance imaging should be considered as it allows simultaneous measurement of ventricular volumes and function and myocardial perfusion, and assessment of possible inflammation and/or fibrosis. Biological variables, such as B-type natriuretic peptides, are highly relevant, valuable markers of global heart involvement in systemic sclerosis and should be considered for screening of patients and/or research purposes.
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Affiliation(s)
- Christophe Meune
- Service de cardiologie, hôpital Cochin, AP-HP, université Paris Descartes, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France.
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Meune C, Avouac J, Wahbi K, Cabanes L, Wipff J, Mouthon L, Guillevin L, Kahan A, Allanore Y. Cardiac involvement in systemic sclerosis assessed by tissue-doppler echocardiography during routine care: A controlled study of 100 consecutive patients. ACTA ACUST UNITED AC 2008; 58:1803-9. [PMID: 18512815 DOI: 10.1002/art.23463] [Citation(s) in RCA: 138] [Impact Index Per Article: 8.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Abstract
OBJECTIVE To assess the prevalence of primary cardiac complications in a large population of patients with systemic sclerosis (SSc), using recently developed echocardiographic techniques. METHODS We prospectively studied 100 consecutive patients (mean +/- SD age 54 +/- 14 years; 86 women) presenting with SSc without pulmonary arterial hypertension or clinical manifestations of heart failure. All patients underwent standard echocardiography, along with measurements of longitudinal velocities by tissue Doppler imaging (TDI) to assess left ventricular (LV) and right ventricular (RV) contractility and LV diastolic function. Results were compared with those in 26 age- and sex-matched healthy controls. RESULTS Patients with SSc had a wider mean left atrial diameter and impaired relaxation compared with the controls. A trend was observed toward a smaller LV ejection fraction (EF) in the patients (mean +/- SD 64.9 +/- 0.6%) than in the controls (67.2 +/- 0.7%), as well as higher pulmonary artery pressure (mean +/- SD 33.3 +/- 0.6 mm Hg versus 30.8 +/- 1.0 mm Hg). LVEF was <55% in 7 patients versus none of the controls. Peak systolic mitral annular velocity as measured by TDI was <7.5 cm/second in 14 patients versus none of the controls (P = 0.040). Mitral annulus early diastolic velocity was <10 cm/second in 30 patients versus 2 of the controls (P = 0.022). Fifteen patients and none of the controls had reduced peak systolic tricuspid annular velocity (P = 0.039). The TDI results correlated with each other, but not with lung abnormalities or other disease characteristics. CONCLUSION Depression of LV and RV systolic and LV diastolic function is common in patients with SSc and is due to primary myocardial involvement. Considering the major contributions of TDI, the addition of this simple technique to standard measurements may improve the detection of heart involvement in patients with SSc.
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Affiliation(s)
- Christophe Meune
- Hôpital Cochin, Université Paris Descartes, Assistance Publique Hôpitaux de Paris, Paris, France
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Abstract
Heart disease is a frequent and often severe feature of systemic sclerosis (scleroderma). Cardiomyopathy, with ventricular diastolic dysfunction and arrhythmias, is the most important form, since it is associated with a very poor prognosis. The current challenge is to define its pattern and identify individuals at risk, but evaluation in vivo may be hard to perform. The aim of this review is to provide an update on the clinical aspects of scleroderma heart disease and the early pivotal role that coronary microcirculation dysfunction plays in its development. A discussion of the diagnostic tools now available for this frequently asymptomatic condition will be provided. Treatment options will be reviewed, even though no cure for systemic sclerosis exists, and the current therapy of diastolic dysfunction remains unsatisfactory.
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Affiliation(s)
- B Marasini
- Rheumatology Unit, Humanitas Clinical Institute, University of Milan, Italy.
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Kawut SM, Taichman DB, Archer-Chicko CL, Palevsky HI, Kimmel SE. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest 2003; 123:344-50. [PMID: 12576350 DOI: 10.1378/chest.123.2.344] [Citation(s) in RCA: 249] [Impact Index Per Article: 11.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/04/2023] Open
Abstract
STUDY OBJECTIVES The goal of this study was to determine whether the survival of patients with pulmonary hypertension related to systemic sclerosis (SScPH) was different from that of patients with other forms of pulmonary arterial hypertension. DESIGN Retrospective cohort study. SETTING Tertiary care medical center. PATIENTS Our cohort was composed of 33 patients with pulmonary hypertension that is sporadic, familial, or related to anorexigen use (PPH) and 22 patients with SScPH who underwent initial pulmonary artery catheterization and vasodilator study at our center between January 1997 and June 2001. MEASUREMENTS AND RESULTS Patients with SScPH had somewhat lower percentage of predicted lung volumes than patients with PPH (total lung capacity, 80% vs 92%; p = 0.06) and had lower percentage of predicted diffusion capacity of the lung for carbon monoxide (42% vs 68%; p = 0.0002). Right atrial pressure, pulmonary artery pressure, and cardiac index were similar between the groups. Patients with SScPH and PPH were treated with usual medical therapies, such as digoxin, warfarin, and continuous IV epoprostenol. Despite these similarities, the risk of death in patients with SScPH was higher than in patients with PPH (unadjusted hazard ratio, 2.9; 95% confidence interval, 1.1 to 7.8; p = 0.03). This increased risk appeared to persist after adjustment for a variety of demographic, hemodynamic, or treatment variables. CONCLUSIONS Despite having similar hemodynamics, patients with SScPH have a higher risk of death than patients with PPH. Future studies of the mechanism and therapy of pulmonary arterial hypertension should focus on the distinctions between the different forms of this disease.
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Affiliation(s)
- Steven M Kawut
- Pulmonary, Allergy, and Critical Care, University of Pennsylvania School of Medicine, Philadelphia, USA.
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Riboldi P, Gerosa M, Luzzana C, Catelli L. Cardiac involvement in systemic autoimmune diseases. Clin Rev Allergy Immunol 2002; 23:247-61. [PMID: 12402411 DOI: 10.1385/criai:23:3:247] [Citation(s) in RCA: 33] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
The heart and the vascular system are frequent and characteristic targets of several systemic autoimmune diseases, in particular Systemic Lupus Erythematosus (SLE), Rheumatoid Arthritis (RA) and Systemic Sclerosis (SSc). In this chapter we review the classic cardiac abnormalities and the more recent data about cardiovascular involvement as part of a major disease complication determining a substantial morbidity and mortality. In addition to the classic cardiac abnormalities involving the heart structures, acute and chronic ischemic heart disease and cerebrovascular accidents are threatening clinical manifestations of SLE and RA associated to an early accelerated atherosclerosis. Immune-mediated inflammation is now recognized as an important factor involved in the pathogenesis of atherosclerosis. Ongoing clinical studies are being devised to find specific risk factors associated with systemic autoimmune diseases and/or treatment regimens. Hopefully, prophylactic measures should be available within the next few years.
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Affiliation(s)
- Piersandro Riboldi
- Department of Internal Medicine, University of Milan, Allergy and Clinical Immunology Unit, IRCCS Istituto Auxologico, Via Ariosto 13, 20145, Milan, Italy.
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Abdelmalek NF, Gerber TL, Menter A. Cardiocutaneous syndromes and associations. J Am Acad Dermatol 2002; 46:161-83; quiz 183-6. [PMID: 11807427 DOI: 10.1067/mjd.2002.120928] [Citation(s) in RCA: 36] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
Abstract
UNLABELLED Multiple cardiocutaneous syndromes and associations are described and reviewed with the emphasis on syndromes that are likely first to be recognized by dermatologists, thereby creating awareness of potential significant cardiovascular disease. Multiple lentigines syndrome, the Carney complex, and Marfan syndrome are examples of syndromes with embryologic and genetic overlap between the cardiac and cutaneous systems. An exhaustive, complete listing of every known disorder or syndrome with combined cardiac and cutaneous findings is beyond the scope of this review. However, all the common associations, both acquired and inherited, including connective tissue, autoimmune, infiltrative, and endocrine disorders are discussed. Because of the serious nature of many of the cardiac defects, early recognition of these conditions by dermatologists and appropriate referrals to colleagues is essential; in addition, when appropriate, genetic counseling is recommended for known heritable disorders. LEARNING OBJECTIVE At the conclusion of this learning activity, participants should be familiar with the embryology of the neural crest, specifically the development of melanocytes and the heart with its vessels, leading to shared abnormalities of the two systems; the major syndromes and associations producing both cutaneous changes, especially of the pigment systems, and cardiovascular abnormalities; the role of the dermatologist in recognizing these changes; and the need to be cognizant and alert in helping screen for associated systemic disease.
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Affiliation(s)
- Nagla F Abdelmalek
- Division of Dermatology and Cardiology, Department of Internal Medicine, Baylor University Medical Center, Dallas, TX, USA
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