REVIEW OF THE LONG-TERM COURSE OF 52 PATIENTS WITH PULMONARY ATRESIA AND VENTRICULAR SEPTAL-DEFECT - ANATOMICAL AND SURGICAL CONSIDERATIONS

This study reviews the long-term course of 52 patients with pulmonary atresia and ventricular septal defect seen in a single institution and followed for a mean period of 8.6 years (range 2 days to 20 years). B efore the first operation, pulmonary blood supply was provided by duct us arteriosus supplying confluent pulmonary arteries in 26 patients (5 0%, group I), and was partially or entirely dependent on systemic coll ateral arteries in the other 26 patients (group II). The angiographic mean ratio of diameters of the Fight and left pulmonary artery/descend ing aorta (McGoon ratio) was significantly lower in group II than in g roup I, 0.76 +/- 0.42 vs 1.04 +/- 0.17 (P=0.006). Severe arborization defects (with fewer than 10 pulmonary vascular segments connected to c entral pulmonary arteries) were present only in group II patients (eig ht patients: 15%), six of whom had congenital absence of the central p ulmonary arteries. Corrective surgery was performed in 23 patients (44 %, 14 in group I, nine in group II). All but one, who died later, had a McGoon ratio greater than or equal to 1 (mean 1.19 +/- 0.18) at time of repair. There was one hospital death (4%) and two late deaths (9%) . All but one of the surviving corrected patients were in functional c lass I or II. Conduit replacement reoperation was performed in three p atients (14%), 6, 10 and 13 years, respectively, after repair. At the end of the study, among the 37 patients (71%) who were alive (17 in gr oup I, 20 in group II), 20 (39%) were corrected (12 in group I, eight in group II), four await corrective surgery, and six (11.5%) are estim ated inoperable (all in group II) because of very hypoplastic or absen t pulmonary arteries. This study confirms the estimated rate in the pu blished literature of long-term survival in patients with pulmonary at resia and ventricular septal defect, and the good results of correctiv e surgery whenever the size and distribution of pulmonary arteries are satisfactory. The problem of very hypoplastic pulmonary arteries and severe arborization defects remains contentious.