Pulmonary artery morphology and hemodynamics in pulmonic valve atresia with ventricular septal defect before and after repair

Abstract Cardiac catheterization and angiography were performed in 22 patients with pulmonic valve atresia and ventricular septal defect to evaluate pulmonary morphology and hemodynamics before and after repair. In 12 of the 22, pulmonic valve atresia and ventricular septal defect were associated with major aortopulmonary collateral arteries, which were ligated in most. Mean postoperative pulmonary artery pressure (PAP) ranged from 9 to 92 mm Hg (mean 28 ± 19) and pulmonary vascular resistance ranged from 1.1 to 35.2 U · m 2 (mean 6.4 ± 8.0). These data correlated (r = 0.89, p 2 was significantly higher in patients with >36 pulmonary artery subsegments connected to the central pulmonary arteries and with a preoperative pulmonary artery area index >0.5 (88%) (p These results indicate that postoperative PAP and pulmonary vascular resistance in patients with pulmonic valve atresia and ventricular septal defect may be predictable when the pulmonary artery area at prebranching and the number of pulmonary artery subsegments connected to the central pulmonary arteries are measured before repair. Early palliation to increase pulmonary artery size and the number of pulmonary artery subsegments connected to the central pulmonary arteries is recommended for obtaining normal pulmonary hemodynamics after repair.