Methods: The study included 71 patients (39 boys, 32 girls; mean age 5.0±4.4 years; range 1 to 29 years) who underwent surgical correction with the transatrial/transpulmonary approach between 2002 and 2005. Perioperative and postoperative data were recorded and evaluated in a prospective fashion. Seven patients had a prior palliative shunt operation. The mean follow-up was 15.2±10.9 months.

Results: Early mortality occurred in four patients: one patient died during surgery; two patients died from sepsis after prolonged mechanical ventilation, and one patient died from multiorgan failure secondary to low cardiac output resulting from intractable junctional tachycardia. Postoperative echocardiographic assessment showed minimal pulmonary insufficiency in 51 patients (76.1%) and mild pulmonary insufficiency in 13 patients (19.4%). Except for two patients, there were no significant right ventricular outflow tract (RVOT) obstruction and residual ventricular septal defect. Only one patient required reoperation in the eighth month due to infective endocarditis. At the end of the follow-up period, all the patients were in NYHA class I (n=66, 98.5%) or class II (n=1, 1.5%). The mean RVOT gradient was 16.3±5.6 mmHg. Tricuspid insufficiency was minimal in 58 patients (86.6%). Only two patients (3.0%) had severe (≥ 3+) tricuspid valve insufficiency. Mild impairment in right ventricular function was detected in only five patients (7.5%), whereas 62 patients (92.5%) had normal right ventricular function.

Results: In terms of postoperative right ventricular function, early results of transatrial/transpulmonary approach are excellent.