Risultati a medio termine dell'approccio integrato all'atresia polmonare con difetto interventricolare e collaterali sistemico-polmonari maggiori.

BACKGROUND: The aim of this study was to validate the preliminary surgical results of the integrated approach to pulmonary atresia, ventricular septal defect (VSD), and multiple aortopulmonary collateral arteries by retrospective analysis of our center experience. METHODS: Between January 1994 and January 2003, 40 patients aged 22 days to 13 years underwent surgery for pulmonary atresia, VSD, and multiple aortopulmonary collaterals. Case selection was based on preoperative calculation of total neopulmonary arterial index (TNPAI), pulmonary arterial index (PAI), and pulmonary arteries-to-collateral arteries lung segment perfusion ratio (Spa:Sca). The decision for a possible VSD closure during one-stage procedures was based on an intraoperative pulmonary flow study. Twenty-seven patients with a TNPAI > or = 150 mm2/m2 underwent primary unifocalization, irrespective of PAI and Spa:Sca. Conversely, 13 patients with a TNPAI <150 mm2/m2 and hypoplastic (PAI <100 mm2/m2) dominant (Spa:Sca > 1) pulmonary arteries received a first-stage right ventricular outflow tract reconstruction, followed by unifocalization and repair (i.e., VSD closure) in 11 cases. RESULTS: Among 38 patients who received total unifocalization, the overall repairability rate was 84% (first instance repairs: n = 29; delayed VSD closure: n = 3; 95% confidence interval 72-96%), with a survival rate at 7.8 years of 83%. Repaired survivors (n = 29) are asymptomatic (n = 25) or mildly symptomatic (n = 4) at a follow-up interval of 43 +/- 30 months, with a 0.48 +/- 0.2 mean hemodynamic right ventricular/left ventricular pressure ratio. CONCLUSIONS: Increasing experience with the integrated approach to pulmonary atresia, VSD, and multiple aortopulmonaty collaterals has confirmed the preliminary results of our surgical series. The pulmonary flow study remains the most accurate intraoperative test for successful management of VSD during unifocalization procedures.