Congenital malformation of the heart associated with congenital anomalies of the urinary tract: Including a syndrome of ventricular septal defect, pulmonary hypertension, mental retardation, and skeletal deformity

Abstract

Review of 26,000 autopsy reports revealed 279 cases in which there were major anomalies of the urinary tract. In 65 of these associated cardiac malformations were found. In 28 there was an isolated ventricular septal defect. Twenty-three of these patients died within the first year, and the remaining 5 reached childhood or adolescence. Two died of congestive heart failure and 3 at operation. All 5 developed skeletal deformity, mental retardation, and showed microscopic evidence of severe pulmonary vascular lesions. The review discloses a relatively high incidence of the association of the ventricular septal defect and major urinary tract abnormality; the relation of such abnormalities to pulmonary lesions remains obscure.