Sudden cardiac death of a 16-year-old boy with Wolff-Parkinson-White syndrome and hypertrophic cardiomyopathy

Abstract

In Europe, 350,000 people die suddenly and unexpectedly every year. The majority of sudden deaths are caused by cardiac alterations and known as sudden cardiac death (SCD). The interpretation of medicolegal findings is especially difficult in those SCDs where there is no morphological correlate and no antemortem electrocardiograms or relevant medical history are available. The Wolff-Parkinson-White (WPW) syndrome, in which an accessory electrical conduction pathway exists between the atrium and the ventricle in addition to the physiological conduction system, is mostly a benign disease of the heart, which may lead to cardiac arrhythmia. A 16-year-old boy with a known history of WPW syndrome since the second year of life had consumed alcohol before death and was found sitting on the toilet fully dressed. The autopsy performed on the deceased 3 days postmortem showed a marked mass increase of the heart with left ventricular concentric hypertrophy and macroscopic signs of cardiomyopathy. In the microscopic examination, the right and left ventricles and the atria were found to have a regular structure with normal vessels. Hematoxylin-eosin (HE) and elastica-van-Gieson (EvG) staining disclosed a thin accessory muscle bridge connecting the left atrium with the left ventricle as a possible morphological correlate of a fatal cardiac arrhythmia.