Inborn heart deformation as a cause of sudden death in a 7-year old girl

Abstract

We report the case of a 7-year old girl who is treated with antibiotics because of an angina tonsillaris. Five days later, vomiting and diarrhea develop and the child is admitted to a hospital. Shortly after admission, cardiac arrest occured, and rescuscitation attempts proved to be unsuccessful. Autopsy shows an inborn heart deformation of the right and also of the left, ventricle: basal trabecular hypertrophy and outflow problems such as a subvalvular pulmonary stenosis in the right ventricle. Additionally, hints for a chronic heart overload were seen in both ventricles. Histologically, multiple necrotic areas in the inner muscle layers in various stages of organization appear besides a multifocal, irregular course of hypertrophic muscle cells as well as fatty inclusions in the myocardial cells. The described heart deformation can be classified nearest as a primary cardiomyopathy with consecutive ventricular noncompaction. It remains questionable, how the girl could reach an age of almost eight years, even without showing signs of cardiac insuffiency