Authors
Gherardo Finocchiaro, Michael Papadakis, Gaia Tanzarella, Harshil Dhutia, Chris Miles, Maite Tome, Elijah R Behr, Sanjay Sharma, Mary N Sheppard
Publication date
2019/2/18
Journal
JACC: Clinical Electrophysiology
Volume
5
Issue
2
Pages
252-254
Publisher
JACC: Clinical Electrophysiology
Description
Hypertrophic cardiomyopathy (HCM) is an inherited cardiomyopathy that is characterized by left ventricular hypertrophy, myocyte disarray at histology, and a propensity for fatal cardiac arrhythmias (1). Sudden cardiac death (SCD) is the most feared complication, particularly among individuals in the second and third decade of life, and several clinical predictors are used to identify high-risk patients who may benefit from an implantable cardioverter-defibrillator (2). Unfortunately, SCD may be the first manifestation of the disease in apparently healthy and asymptomatic individuals, including athletes.
The aim of the study was to report the characteristics and circumstances of death in a large cohort of decedents of SCD with a diagnosis of HCM at autopsy. We reviewed a database of 5,100 consecutive cases of SCD referred to our specialist cardiac pathology center between January 1994 and March 2017 and identified …
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