Authors
Chris Miles, Gherardo Finocchiaro, Michael Papadakis, Belinda Gray, Joseph Westaby, Bode Ensam, Joyee Basu, Gemma Parry-Williams, Efstathios Papatheodorou, Casey Paterson, Aneil Malhotra, Jan Lukas Robertus, James S Ware, Stuart A Cook, Angeliki Asimaki, Adam Witney, Irina Chis Ster, Maite Tome, Sanjay Sharma, Elijah R Behr, Mary N Sheppard
Publication date
2019/1/31
Journal
Circulation
Publisher
American Heart Association
Description
Background
Arrhythmogenic cardiomyopathy (ACM) is an inherited heart muscle disorder characterized by myocardial fibrofatty replacement and an increased risk of sudden cardiac death (SCD). Originally described as a right ventricular disease, ACM is increasingly recognized as a biventricular entity. We evaluated pathological, genetic, and clinical associations in a large SCD cohort.
Methods
We investigated 5205 consecutive cases of SCD referred to a national cardiac pathology center between 1994 and 2018. Hearts and tissue blocks were examined by expert cardiac pathologists. After comprehensive histological evaluation, 202 cases (4%) were diagnosed with ACM. Of these, 15 (7%) were diagnosed antemortem with dilated cardiomyopathy (n=8) or ACM (n=7). Previous symptoms, medical history, circumstances of death, and participation in competitive sport were recorded. Postmortem genetic testing …
Scholar articles
C Miles, G Finocchiaro, M Papadakis, B Gray… - Circulation, 2019