Review of the long-term course of 52 patients with pulmonary atresia and ventricular septal defect

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.

Staged repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: Experience with 104 patients

“…The overall mortality rate observed in our series is comparable to that noted in previous studies. [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] The pRV/pLV is widely regarded as a surrogate measure of functional status after complete anatomic repair of this lesion. The pRV/pLV observed in our study is also comparable to that reported in other large series.…”

“…The pRV/pLV observed in our study is also comparable to that reported in other large series. [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] We believe that this favorable pRV/pLV, particularly in patients who had extremely hypoplastic true pulmonary arteries with relatively few MAPCAs, is the result of the aggressive approach used to encourage growth of the true pulmonary arteries and aggressive recruitment of as many MAPCAs as possible in the repair, resulting in a larger effective pulmonary vasculature. In the univariate analysis we found that unifocalization of fewer than 3 MAPCAs in the repair was significantly associated with an increased likelihood of mortality and pRV/pLV Ͼ 0.55 (P Ͻ .05).…”

Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: collateral vessel disease burden and unifocalisation strategies

“…To date, several classification systems have been proposed. The Nakata Index 15 , 16 and McGoon ratio 17 , 18 have been used to evaluate the central/intrapericardial pulmonary arteries. The total neopulmonary artery index, a combined index for all major aortopulmonary collaterals and the central pulmonary artery, has been shown to correlate with postoperative right ventricle/left ventricle pressure ratio 9 .…”

Tetralogy of Fallot

“…The size of the branch pulmonary arteries is inversely proportional to the extent of MAPCAs and presence of a ductus arteriosus [102][103][104][105]. The angiographically determined Nakata index predicts good outcomes of complete repair of TOF with pulmonary artery hypoplasia if the index is greater than 100 mm 2 /body surface area (BSA) [106].…”