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Review
. 2024 Jul 18;12(7):1602.
doi: 10.3390/biomedicines12071602.

Cardiomyopathy and Sudden Cardiac Death: Bridging Clinical Practice with Cutting-Edge Research

Raffaella Mistrulli  1   2 , Armando Ferrera  1 , Luigi Salerno  1 , Federico Vannini  1 , Leonardo Guida  1 , Sara Corradetti  1   2 , Lucio Addeo  2   3 , Stefano Valcher  2   4 , Giuseppe Di Gioia  5 , Francesco Raffaele Spera  1 , Giuliano Tocci  1 , Emanuele Barbato  1
Affiliations
Review

Cardiomyopathy and Sudden Cardiac Death: Bridging Clinical Practice with Cutting-Edge Research

Raffaella Mistrulli et al. Biomedicines. .

Abstract

Sudden cardiac death (SCD) prevention in cardiomyopathies such as hypertrophic (HCM), dilated (DCM), non-dilated left ventricular (NDLCM), and arrhythmogenic right ventricular cardiomyopathy (ARVC) remains a crucial but complex clinical challenge, especially among younger populations. Accurate risk stratification is hampered by the variability in phenotypic expression and genetic heterogeneity inherent in these conditions. This article explores the multifaceted strategies for preventing SCD across a spectrum of cardiomyopathies and emphasizes the integration of clinical evaluations, genetic insights, and advanced imaging techniques such as cardiac magnetic resonance (CMR) in assessing SCD risks. Advanced imaging, particularly CMR, not only enhances our understanding of myocardial architecture but also serves as a cornerstone for identifying at-risk patients. The integration of new research findings with current practices is essential for advancing patient care and improving survival rates among those at the highest risk of SCD. This review calls for ongoing research to refine risk stratification models and enhance the predictive accuracy of both clinical and imaging techniques in the management of cardiomyopathies.

Keywords: cardiomyopathies; cardiovascular prevention; risk stratification; sudden cardiac death.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Comparison between European and American guidelines for SCD prevention in HCM patients. CMR: cardiac magnetic resonance; EF: ejection fraction; FH: family history of sudden cardiac death; LVH: left ventricular hypertrophy; LGE: late gadolinium enhancement; and NSVT: non-sustained ventricular tachycardia.
Figure 2
Figure 2
Established and emerging risk factors for sudden cardiac death in dilated cardiomyopathy. LMNA, lamin A/C; DSP, desmoplakin; PLN, phospholamban; FLNC, filamin C; RBM20, RNA-binding motif protein 20; DES, desmin; TTN, titin; LGE, late gadolinium enhancement; ECG, electrocardiogram; NSVT: non-sustained ventricular tachycardia; VE, premature ventricular complexes; PES, programmed electrical stimulation; LVEF, left ventricular ejection fraction; NT-proBNP, N-terminal pro-B-type natriuretic peptide; and SCD, sudden cardiac death.
Figure 3
Figure 3
Established and emerging risk factors for sudden cardiac death in arrhythmogenic right ventricular cardiomyopathy. DSP, desmoplakin; TMEM43, transmembrane protein 43; LMNA, lamin A/C; PLN, phospholamban; CMR, cardiac magnetic resonance; LV, left ventricular; RV, right ventricular; ECG, electrocardiogram; NSVT; non-sustained ventricular tachycardia; VE, premature ventricular complexes; VT, ventricular tachycardia; and PES, programmed electrical stimulation.
Figure 4
Figure 4
Overlapping genetic background in cardiomyopathies. * The list is not exhaustive. ARVC, arrhythmogenic right ventricular cardiomyopathy; DCM, dilated cardiomyopathy; DES, desmin; DSG, desmoglein; DSP, desmoplakin; FLNC, filamin C; JUP, plakoglobin; LMNA, lamin A/C; NDLVC, non-dilated left ventricular cardiomyopathy; PKP, plakophilin; PLN, phospholamban; TMEM43, transmembrane protein 43; RBM20, RNA-binding motif protein 20; and SCN5A, sodium channel protein type 5.
See this image and copyright information in PMC

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