PULMONARY-ARTERY MORPHOLOGY AND HEMODYNAMICS IN PULMONIC VALVE ATRESIA WITH VENTRICULAR SEPTAL-DEFECT BEFORE AND AFTER REPAIR

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 . m2 (mean 6.4 +/- 8.0). These data correlated (r = 0.89, p < 0.001). The number of pulmonary artery subsegments connected to the central pulmonary arteries was 22 to 42 (mean 38 +/- 6). Univariate analysis revealed that the mean postoperative PAP correlated with the number of pulmonary artery subsegments connected to the central pulmonary arteries (r = -0.81, p < 0.001), with mean preoperative PAP (r = 0.79, p < 0.001), with the postoperative pulmonary artery area index of the right and left pulmonary arteries at prebranching (r = -0.76, p < 0.001), and with the sum of the pulmonary artery areas after branching (r = -0.69, p < 0.005). Pulmonary vascular resistance correlated with the number of pulmonary artery subsegments connected to the central pulmonary arteries (r = -0.85, p < 0.001), with the mean preoperative PAP (r = 0.79, p < 0.001), with the sum of the pulmonary artery areas after branching (r = -0.73, p < 0.001), and with the postoperative pulmonary artery area index (r = -0.70, p < 0.001). The incidence of pulmonary vascular resistance being < 3 U . m2 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 < 0.01). 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.