Two patients with hypoplastic right ventricle who died following closure of an associated ventricular septal defect are described. Both cases presented with large left-to-right shunts and pulmonary hypertension. In each case, the diagnosis of an endocardial cushion defect was suggested by the ECG. The presence of the hypoplastic right ventricle was not suspected on electrocardiographic or vectorcardiographic grounds, since both showed the presence of right ventricular forces probably generated from the outflow portion of the right ventricle. Thoracic roentgenograms were not diagnostically helpful. Since the condition closely resembles endocardial cushion defect unaccompanied by incompetence of the atrioventricular valves, or the occasional case of infracristal ventricular septal defect with an electrocardiogram similar to the aforementioned condition, the use of selective right ventricular angiography under these circumstances may be of diagnostic assistance in identifying the presence of a hypoplastic right ventricle.
Bibliographical noteFunding Information:
From the Department of Pathology, The Charles T. Miller Hospital, St. Paul, Minnesota, Pediatrics, Surgery, and Pathology, University of Minnesota, Minneapolis, Minn. This study was supported by the United States Public Health Service Research Grant 5 ROi HE-05694. Research Training Grant 5 Tl HE 5570, and Research Grant HE-00830, from the National Heart Institute, the Max Baer Heart Fund of the Fraternal Order of the Eagles, and the Maria and Joseph Gales Ramsay, III, Foundation. Received for publication July 17, 1967.