A strange complex of diffuse congenital cardiovascular disease and cardiomyopathy, with localised myocardial calcification.
This unique case is of a man, followed clinically since infancy, who had a ventricular septal defect which closed spontaneously, a small arterial duct, and a minor degree of aortic coarctation, all without obvious symptoms. He later developed progressive cardiac failure which was attributed to some obscure form of congenital cardiomyopathy. He died at the age of 45 years. Necropsy showed a grossly abnormal arrangement of ventricular myocardial fascicles and bands, with absence of the papillary muscles causing tricuspid and mitral regurgitation. The various malformations are considered to be a gross example of a diffuse congenital cardiovascular disease complex, to the best of our knowledge previously undescribed.
Gerlis
L M
LM
Department of Paediatric Cardiac Morphology, The Royal Brompton Hospital, Imperial College School of Medicine, London, UK.
Somerville
J
J
D002114:Calcinosis / Q000473:pathology*; D009202:Cardiomyopathies / Q000473:pathology*; D017809:Fatal Outcome; D006330:Heart Defects, Congenital / Q000150:complications / Q000473:pathology*; D006333:Heart Failure / Q000209:etiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology; D010210:Papillary Muscles / Q000002:abnormalities