Unclassified familial cardiomyopathy with ventricular dysrhythmia.
The case is described of a boy with some right ventricular dysplasia and episodes of ventricular tachycardia of left bundle branch block pattern who had symptoms from the age of 1 month. Angiography and cardiac biopsy demonstrated major involvement of the left ventricular myocardium. A sister of the patient presented at the age of 2 months with predominantly left ventricular cardiomyopathy; clinical signs and angiography suggested the presence of right ventricular dysplasia as well. She died suddenly at the age of 9 years. Her brother, now aged 14 years, is being treated with antiarrhythmic drugs. The hypothesis of this cardiomyopathy being a variant of "arrhythmogenic right ventricular dysplasia syndrome" is discussed.
Department of Pediatric Cardiology, Free University Hospital, Amsterdam, The Netherlands.
R J
RJ
Moene
M A
MA
Sobotka
B
B
Buis
G T
GT
Bots
N J
NJ
Elzenga
D002037:Bundle-Branch Block / Q000503:physiopathology; D009202:Cardiomyopathies / Q000235:genetics*; D005260:Female; D006330:Heart Defects, Congenital / Q000235:genetics; D006352:Heart Ventricles / Q000503:physiopathology; D006801:Humans; D007223:Infant; D008297:Male; D013610:Tachycardia / Q000235:genetics*