Total congenital atrioventricular blockage in a patient with endocardial fibroplastosis and the persistence of arterial duct. Diagnosis in utero.

Abstract

The authors present the case of a rare association of several congenital heart malformations: a complete A-V block, an endocardial fibroelastosis and a patent ductus arteriosus. The complete atrioventricular block had been suspected during pregnancy by the low heart rate of the fetus. It was confirmed on the first day of life with electrocardiogram. The heart rate was extremely low, with a ventricular rate of 25 beats per minute. Endocardial fibroelastosis was confirmed by the macro and microscopical examination. Both ventricles and the left auricle were involved. It was more marked on the left ventricle. The size of the heart was four times the normal size; its weight was lightly increased. The myocardium of the left ventricle was twice as thick as normal and its endocardium ten times as thick as normal. The ductus arteriosus was patent. The child was born prematurely and lived 36 days. She died as a result of extreme dehydration.