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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.

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