2021
DOI: 10.1016/j.hroo.2021.10.009
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Ventricular arrhythmia management in patients with genetic cardiomyopathies

Abstract: Genetic cardiomyopathies are associated with increased risk for cardiac arrhythmias and sudden cardiac death. The management of ventricular arrhythmias (VAs) in patients with these conditions can be nuanced due to particular disease-based considerations, yet data specifically addressing management in these patients are limited. Here we describe the current evidence-based approach to the management of ventricular rhythm disorders in patients with genetic forms of cardiomyopathy, namely, hypertrophic cardiomyopa… Show more

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“…In addition, in ACM, specific mutations may correlate with earlier manifestation phenotypes and may influence risk stratification and management [ 74 ]. Considering the prognostic profile of gene positivity, the identification of some pathogenic mutations may help decision making about primary ICD implantation [ 99 ]. With regard to non-desmosomal gene defects, the TMEM43 p.S358L founder mutation is almost fully penetrant and highly lethal among male carriers and should be to be considered by itself an indication of prophylactic ICD [ 100 ].…”
Section: The World Of Acm and Dcm: How “Complex Imaging” (Electroanat...mentioning
confidence: 99%
“…In addition, in ACM, specific mutations may correlate with earlier manifestation phenotypes and may influence risk stratification and management [ 74 ]. Considering the prognostic profile of gene positivity, the identification of some pathogenic mutations may help decision making about primary ICD implantation [ 99 ]. With regard to non-desmosomal gene defects, the TMEM43 p.S358L founder mutation is almost fully penetrant and highly lethal among male carriers and should be to be considered by itself an indication of prophylactic ICD [ 100 ].…”
Section: The World Of Acm and Dcm: How “Complex Imaging” (Electroanat...mentioning
confidence: 99%