Incidence, risk assessment and prevention of sudden cardiac death in cardiomyopathies

ABSTRACT Cardiomyopathies are a significant contributor to cardiovascular morbidity and mortality, mainly due to the development of heart failure and increased risk of sudden cardiac death (SCD). Despite improvement in survival with contemporary treatment, SCD remains an important cause of mortality...

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Published in:European journal of heart failure 2023-12, Vol.25 (12), p.2144-2163
Main Authors: Polovina, Marija, Tschöpe, Carsten, Rosano, Giuseppe, Metra, Marco, Crea, Filippo, Mullens, Wilfried, Bauersachs, Johann, Sliwa, Karen, Boer, Rudolf A., Farmakis, Dimitrios, Thum, Thomas, Corrado, Domenico, Bayes‐Genis, Antoni, Bozkurt, Biykem, Filippatos, Gerasimos, Keren, Andre, Skouri, Hadi, Moura, Brenda, Volterrani, Maurizio, Abdelhamid, Magdy, Ašanin, Milika, Krljanac, Gordana, Tomić, Milenko, Savarese, Gianluigi, Adamo, Marianna, Lopatin, Yuri, Chioncel, Ovidiu, Coats, Andrew J.S., Seferović, Petar M.
Format: Article
Language:English
Subjects:
Arrhythmia
Arrhythmias, Cardiac - complications
Arrhythmias, Cardiac - epidemiology
Arrhythmogenic right ventricular cardiomyopathy
Cardiomyopathies - complications
Cardiomyopathies - epidemiology
Death, Sudden, Cardiac - epidemiology
Death, Sudden, Cardiac - etiology
Death, Sudden, Cardiac - prevention & control
Dilated cardiomyopathy
Heart Failure - complications
Humans
Hypertrophic cardiomyopathy
Hypertrophy, Left Ventricular - complications
Incidence
Medicin och hälsovetenskap
Non‐dilated left ventricular cardiomyopathy
Primary prevention
Restrictive cardiomyopathy
Risk Assessment
Risk Factors
Risk stratification
Sudden cardiac death
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Summary:ABSTRACT Cardiomyopathies are a significant contributor to cardiovascular morbidity and mortality, mainly due to the development of heart failure and increased risk of sudden cardiac death (SCD). Despite improvement in survival with contemporary treatment, SCD remains an important cause of mortality in cardiomyopathies. It occurs at a rate ranging between 0.15% and 0.7% per year (depending on the cardiomyopathy), which significantly surpasses SCD incidence in the age‐ and sex‐matched general population. The risk of SCD is affected by multiple factors including the aetiology, genetic basis, age, sex, physical exertion, the extent of myocardial disease severity, conduction system abnormalities, and electrical instability, as measured by various metrics. Over the past decades, the knowledge on the mechanisms and risk factors for SCD has substantially improved, allowing for a better‐informed risk stratification. However, unresolved issues still challenge the guidance of SCD prevention in patients with cardiomyopathies. In this review, we aim to provide an in‐depth discussion of the contemporary concepts pertinent to understanding the burden, risk assessment and prevention of SCD in cardiomyopathies (dilated, non‐dilated left ventricular, hypertrophic, arrhythmogenic right ventricular, and restrictive). The review first focuses on SCD incidence in cardiomyopathies and then summarizes established and emerging risk factors for life‐threatening arrhythmias/SCD. Finally, it discusses validated approaches to the risk assessment and evidence‐based measures for SCD prevention in cardiomyopathies, pointing to the gaps in evidence and areas of uncertainties that merit future clarification. Sudden cardiac death in cardiomyoptahies: incidence, risk factors and prevention. AV, atrioventricular; CMR, cardiac magnetic resonance; CRT, cardiac resynchronization therapy; CV, cardiovascular; FLNC, filamin C; GDMT, guideline‐directed medical therapy; HCM, hypertrophic cardiomyopathy; HFrEF, heart failure with reduced ejection fraction; ICD, implantable cardioverter‐defibrillator; LGE, late gadolinium enhancement; LMNA, lamin A/C; LV, left ventricular; LVEF, left ventricular ejection fraction; NSVT, non‐sustained ventricular tachycardia; NYHA, New York Heart Association; OMT, optimal medical therapy; PES, programmed electrical stimulation; PLN, phospholamban; RBM20, RNA‐binding motif protein 20; RV, right ventricular; SCD, sudden cardiac death; VT, ventricular tachycardia. *Syncop
ISSN:1388-9842
1879-0844
1879-0844
DOI:10.1002/ejhf.3076