Title : Predictors of sudden death in congenital arrhythmogenic syndromes

Background: Genetic heart diseases are common causes of sudden cardiac death (SCD) in young individuals, and are typically divided into inherited cardiomyopathies and primary electrical heart diseases. Hypertrophic cardiomyopathy (HCM) and arrhythmogenic cardiomyopathy (ACM) are both cardiomyopathies that increase the risk of SCD.

Objective: To define risk predictors of SCD. Primary electrical diseases that are more commonly seen in clinical practice include Brugada syndrome (BrS) and long QT syndrome (LQTS). Risk stratification for SCD is a must in managing patients with these genetic heart diseases. Numerous risk factors have been identified, and risk prediction models have been developed to estimate the risk of sudden cardiac death (SCD). These tools assist clinicians and patients in making decisions about the use of prophylactic implantable cardioverter-defibrillators (ICDs).

Conclusions: This paper examines the current literature on risk stratification in ARVC and other ACMs, such as BrS and LQTS, and summarizes current recommendations for using ICD.

Samir Morcos Rafla, MB, ChB (Hon.), PhD, FACC, FESC, FHRS, Professor Emeritus of Cardiology Alexandria University, Dr. Rafla is a Fellow of the American College of Cardiology (FACC), an Emeritus Fellow of the European Society of Cardiology (FESC), and a Fellow of the Heart Rhythm Society (FHRS). He is also a member of the European Heart Rhythm Association (EHRA) and a Senior Member of the Egyptian Society of Cardiology. He completed a research fellowship at the Cleveland Clinic Foundation in Ohio, USA (1983–1984), where he further advanced his expertise in cardiac electrophysiology. Dr. Rafla’s primary areas of expertise include cardiac electrophysiology and echocardiography. He has authored four medical books and published more than 100 scientific papers in peer-reviewed journals. His work has received over 540 citations, with an H-index of 7 according to Google Scholar and ORCID. Awards: Award for Scientific Encouragement, Alexandria University (1994), Award for Scientific Distinction, Egyptian Medical Syndicate (February 2008). Research Profiles: ORCID: https://orcid.org/0000-0001-8688-6532