“Grown-Up” Congenital Heart Disease and Sudden Death in a Medical Examiner’s Population*

Abstract

Despite advances in the management of congenital heart disease (CHD), children with CHD who survive into adulthood are at increased risk of sudden death. Sudden death may also be the initial presentation of undiagnosed CHD in some adults. This retrospective descriptive study outlines the spectrum of CHD presenting as sudden death in adults in a medical examiner's population. Despite its rarity (0.2% of all cases investigated between 1991 and 2007), CHD remains an important cause of sudden cardiac death to be recognized at adult autopsy. Bicuspid aortic valve and anomalous coronary anomalies were the most common malformations, comprising 36.9% and 26.2% of cases, respectively. However, a wide spectrum of simple to complex malformations can be seen, with or without prior surgery, and over a wide age spectrum. Once solely a pediatric entity, CHD is now "grown-up" and will likely be diagnosed by forensic pathologists with increased frequency in the future.