title: Review of the long-term course of 52 patients with pulmonary atresia and ventricular septal defect. Anatomical and surgical considerations.
abstract: 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). Before the first operation, pulmonary blood supply was provided by ductus arteriosus supplying confluent pulmonary arteries in 26 patients (50%, group I), and was partially or entirely dependent on systemic collateral arteries in the other 26 patients (group II). The angiographic mean ratio of diameters of the right and left pulmonary artery/descending aorta (McGoon ratio) was significantly lower in group II than in group 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 central pulmonary arteries) were present only in group II patients (eight patients: 15%), six of whom had congenital absence of the central pulmonary 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 > or = 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 class I or II. Conduit replacement reoperation was performed in three patients (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 group 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 estimated inoperable (all in group II) because of very hypoplastic or absent pulmonary arteries. This study confirms the estimated rate in the published literature of long-term survival in patients with pulmonary atresia and ventricular septal defect, and the good results of corrective surgery whenever the size and distribution of pulmonary arteries are satisfactory. The problem of very hypoplastic pulmonary arteries and severe arborization defects remains contentious.
authors.affiliation: Hôpital Cardiologique du Haut-Lévêque, Université de Bordeaux II, Pessac, France.
authors.forename: A
authors.initials: A
authors.lastname: Hadjo
authors.forename: M
authors.initials: M
authors.lastname: Jimenez
authors.forename: E
authors.initials: E
authors.lastname: Baudet
authors.forename: X
authors.initials: X
authors.lastname: Roques
authors.forename: N
authors.initials: N
authors.lastname: Laborde
authors.forename: S
authors.initials: S
authors.lastname: Srour
authors.forename: C
authors.initials: C
authors.lastname: Surrel
authors.forename: A
authors.initials: A
authors.lastname: Choussat
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006345:Heart Septal Defects, Ventricular / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D008137:Longitudinal Studies; D008297:Male; D010166:Palliative Care; D018633:Pulmonary Atresia / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D012086:Reoperation