Primary Central Pulmonary Artery Plasty for Single Ventricle With Ductal-Associated Pulmonary Artery Coarctation

Background Infants with a single ventricle who require a modified Blalock-Taussig shunt can have stenosis at the ductal insertion site on the pulmonary artery (PA). We hypothesized that primary PA plasty in the first palliation would limit PA stenosis and unbalanced pulmonary perfusion, and thereby facilitate safe accomplishment of the next operation. Methods From 1998 to 2012, functional single-ventricle patients with pulmonary atresia were managed as follows: initial operative strategy based on midline approach, using cardiopulmonary bypass, and central PA plasty (resection of all ductal tissue). All patients underwent catheter study and lung perfusion test before bidirectional cavopulmonary shunt (BCPS). Results Forty consecutive patients (heterotaxy, n = 27; others, n = 13) were enrolled in this study. Seven patients (17.5%) had repair of obstructed total anomalous pulmonary venous drainage, and 4 (10%) had repair of common atrioventricular valve at the first palliative operation. Fifteen patients (37.5%) underwent first palliation in the neonatal period. The mean PA index (mm2 /m2 ) before BCPS was 230.7 ± 101.7. No pulmonary stenosis was detected before BCPS. Pulmonary artery diameter ratio (nonshunt side/shunt side) was 0.93 ± 0.25; pulmonary lung perfusion ratio (nonshunt side/shunt side) was 0.9 ± 0.2. A multivariate analysis identified persistent total anomalous pulmonary venous drainage (extracardiac type; p = 0.002) as a factor associated with unbalanced branch pulmonary arteries at BCPS. Thirty-five patients underwent BCPS, and 31 underwent Fontan procedures. There were 4 early deaths and 4 late deaths. Conclusions The strategies we have employed facilitate safe accomplishment of the next-stage operation in diverse anatomic groups, with avoidance of unbalanced pulmonary vascular bed.