Premium
Ebstein Anomaly and Sudden Childhood Death
Author(s) -
Freeman Amanda,
Byard Roger W.
Publication year - 2018
Publication title -
journal of forensic sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.715
H-Index - 96
eISSN - 1556-4029
pISSN - 0022-1198
DOI - 10.1111/1556-4029.13652
Subject(s) - medicine , autopsy , ventricle , tricuspid valve , sudden death , anatomy , dysplasia , cardiology , tetralogy of fallot , heart disease
A 13‐year‐old girl is reported who died suddenly and unexpectedly in her sleep from previously undiagnosed Ebstein anomaly. At autopsy, there was dilatation of the right atrium with marked dilatation of the right auricle and apical displacement of the tricuspid valve into the right ventricular cavity with atrialization of the upper portion of the right ventricle. There were also prominent dysplastic changes in both the septal and posterior leaflets of the tricuspid valve with thickening of the valve and fusion of leaflets to the wall of the ventricle. Histology of the myocardium showed focal, minor microscopic areas of interstitial fibrosis with marked fibrous dysplasia and thickening of the tricuspid valve. Lethal arrhythmias occur in this condition because of the geographical relationship of the conduction system to the abnormal anatomical structures. As adolescents who died suddenly are often minimally symptomatic, cases will rarely present de novo to forensic autopsy.