Abstract
This report describes a neonate with congenital partial absence of the pericardium associated with hypoplastic left heart syndrome. The patient presented with severe heart failure caused by a hypoplastic left ventricle. The infant died at 15 hours of age because of intractable congestive heart failure. Necropsy revealed, in addition to a hypoplastic left ventricle, a transverse biventricular constriction suggestive of partial absence of the pericardium. In addition to cardiovascular malformations, a left hydronephrosis and a bicornuate uterus was found. We report a rare clinical entity and review the literature.
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