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[Morphology of right ventricle and hemodynamics in pulmonary atresia with intact ventricular septum].

Cardiac catheterization and angiography were performed in 31 patients with PA/IVS to evaluate the morphology of RV and hemodynamics. The age of patients ranged from 1 day to 39 month (mean 4.7 month). The patients were divided into three groups: mild (19.4%), moderate (64.5%) and severe (16.1%) RV hypoplasia, according to the degree of tricuspid valve and right ventricular hypoplasia. Seven of the 31 patients (22.6%) had right ventricular myocardial sinusoid-coronary artery fistula. The right and left ventricular pressures in 26 patients were measured simultaneously. The right ventricular pressures were supra-systemic in 18 patients (69.2%), systemic in 6 (23.1%), less than systemic pressures in 2 (7.7%); the others were PA/IVS with ASD(6 patients), PFO (25), PDA (25). Palliative and definitive operations were performed on 22 patients. The results show that determining the morphology of RV and the hemodynamics before operation is helpful in selecting appropriate operation for lowering the operative mortality.