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...

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Bibliographic Details
Published in:Pediatric cardiology 1987-01, Vol.8 (3), p.177-180
Main Authors: Moene, R J, Sobotka, M A, Buis, B, Bots, G T, Elzenga, N J
Format: Article
Language:English
Subjects:
Bundle-Branch Block - physiopathology
Cardiomyopathies - genetics
Female
Heart Defects, Congenital - genetics
Heart Ventricles - physiopathology
Humans
Infant
Male
Tachycardia - genetics
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Summary: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.
ISSN:0172-0643
1432-1971
DOI:10.1007/BF02263449