Long Term Outcome of Pulmonary Atresia with Major Aorto-Pulmonary Collaterals: Data from a Pooled Analysis

Prognosis of Tetralogy of Fallot and Pulmonary atresia with MAPCAs is variable. We aimed to assess the outcome of patients who had undergone repair of TOF and PA with MAPCAs by different institutions and approaches, pooling together data on mortality and reinterventions. Abstracts were screened and full text papers were examined retrieving data on mortality, reinterventions and percentage of complete repair. Variables abstracted from the studies were pooled together and compared among different strategies. 28 papers out of 124 were selected, accounting for 1983 patients. 704 patients had undergone single stage unifocalization, 544 recruitment of PAs, 645 patients multistage and combined approaches. Median follow up was 49 months (36-70). 93 deaths occurred in the first month (5.4 %): 38 in group one (5.8%), 10 in group 2 (2.3%) and 45 in group 3 (8.6%), p<0.0001. At last follow up a total of 305 deaths occurred (16%) and did not differ among the three groups: 0.21 (0.15-0.25), 0.18 (0.07-0.26), 0.32 (0,13-0.38) deaths/100patients year, respectively p=0.43. 465 out of 969 patients, underwent at least one re-intervention (47.9%). Reintervention rate was similar among the three subsets and inversely correlated with the volume of institution (r= -0.45, p= 0.04). Although strategies of pulmonary atresia with MAPCAs repair are based on contrasting philosophies and extreme anatomic varibility, pooling together data from different studies, outcomes do not differ significantly and are characterized by a high rate of reinterventions. The latter appears to be significantly influenced by the volume of patients.