Palliative right ventricular outflow tract construction for patients with pulmonary atresia, ventricular septal defect, and hypoplastic pulmonary arteries.

From December, 1977, through June, 1981, 15 patients with pulmonary atresia, ventricular septal defect, and hypoplastic pulmonary arteries underwent a palliative right ventricular outflow tract construction. In seven (Group A) this was the first surgical procedure, whereas in the remaining eight (Group B) previous systemic-pulmonary artery anastomoses had been performed. A high morbidity of the pulmonary arteries was present in Group B, resulting in part from the previous shunting procedures. The left pulmonary artery was severely hypoplastic in five patients; the right was hypoplastic in seven of the eight patients and was severely stenotic or atretic in three. All the patients in Group A survived the palliative right ventricular outflow tract reconstruction, and only one in Group B died. Almost all patients had an increase in the aortic PO2 and oxygen saturations after palliative right ventricular outflow tract construction, with a concomitant fall in hemoglobin concentration. Three of the five patients in Group A who underwent postoperative angiography were found to have proximal left pulmonary artery stenosis, and three had lesser involvement of the proximal right pulmonary artery as well. Three of the six patients in Group B who were studied postoperatively also had severe stenosis or proximal atresia of the right pulmonary artery, and three patients had stenosis and hypoplasia of the left pulmonary artery as well, two severe. Thus in no patient in this series were normal pulmonary arteries achieved by palliative right ventricular outflow tract construction will provide the potential for ultimate complete repair. The rather constant finding of severe proximal left pulmonary artery stenosis and hypoplasia in Group A is disappointing.