| Literature DB >> 28843747 |
Hae Yoon Grace Choung1, Monika Vyas2, Daniel Jacoby3, Brian West4.
Abstract
A 26year old east African professional athlete presented to the obstetric clinic for a routine visit at 36 weeks gestation. She had a history of Right Ventricular Outflow Tract - Ventricular Tachycardia (RVOT-VT) with an episode of cardiac arrest in the past, and had been treated with ablation 4 years earlier. Her current visit was uneventful, her pregnancy progressing normally. Following the visit she went to a local restaurant where she suffered a cardiac arrest that was unresponsive to therapy. Chest compressions were continued from the time of her collapse until an emergency caesarian section was performed, delivering a healthy female infant. At autopsy a focal area of subtle pallor and myocardial thinning was present at the apex of the right ventricle. Histology showed myocyte degeneration and loss with focal full thickness replacement of myocardium by adipose tissue, consistent with the fatty form of arrhythmogenic right ventricular cardiomyopathy (ARVC). Molecular studies revealed a variant of unknown significance in the MYBPC3 gene, but no variant known to be associated with ARVC. In view of the subtlety of the lesion on gross examination this diagnosis could have been easily missed, emphasizing the importance of performing histologic examination of subtle gross cardiac lesions.Entities:
Keywords: ARVC; Arrhythmogenic Right Ventricular Cardiomyopathy; Athletes; Cardiomyopathy; Sudden Death
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Year: 2017 PMID: 28843747 DOI: 10.1016/j.prp.2017.07.015
Source DB: PubMed Journal: Pathol Res Pract ISSN: 0344-0338 Impact factor: 3.250