Increasing experience with integrated approach to pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries

Objectives: To validate the preliminary surgical results of ‘integrated approach’ to pulmonary atresia, ventricular septal defect (VSD), and multiple aortopulmonary collateral arteries by retrospective analysis of our center experience. Methods: Between 01/94 and 03/02, 37 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-five patients with a TNPAI equal to or greater than 150 mm2/m2 underwent primary unifocalization, irrespective of PAI and Spa:Sca. Conversely, 12 patients with a TNPAI less than 150 mm2/m2 and hypoplastic (PAI less than 100 mm2/m2) dominant (Spa:Sca greater than 1) pulmonary arteries received a first-stage right ventricular outflow tract reconstruction, followed by unifocalization and repair (i.e. VSD closure) in nine cases. Results: Among 34 patients who received total unifocalization, the overall repairability rate was 85% (first instance repairs: <f>n=27</f>; delayed VSD closure: <f>n=2</f>; 95% confidence interval, CI: 73–97%), with a survival rate at 7 years of 81%. Repaired survivors (<f>n=26</f>) are asymptomatic (<f>n=22</f>) or mildly symptomatic (<f>n=4</f>) at a follow-up interval of 43±28 months, with a 0.48±0.2 mean haemodynamic right ventricular/left ventricular pressure ratio, whereas palliated ones are waiting for either repair (<f>n=3</f>) or catheter study (<f>n=2</f>). Analysis of results has shown the following: (1) 100% (34/34 cases) feasibility of one-stage unifocalization in patients with a preoperative TNPAI equal to or greater than 150 mm2/m2, whereas combined repairability rate was 79% only (95% CI: 65–93%); (2) 100% (12/12 cases) fulfillment of criteria for second-stage repairability (acquired TNPAI greater than 150 mm2/m2) in all patients treated with right ventricular outflow tract reconstruction; and (3) 93% (95% CI: 83–100%) overall accuracy of intraoperative flow study in predicting either postrepair mean pulmonary arterial pressure (VSD closed: <f>n=23</f>) or balanced pulmonary to systemic blood flow ratio (VSD left open: <f>n=4</f>). Conclusions: Increasing experience with ‘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 [Copyright &y& Elsevier]