نبذة مختصرة : Objectives: To determine the safety and efficacy of right ventricle to pulmonary artery connection and simultaneous treatment of major aortopulmonary collaterals for rehabilitation of the hypoplastic pulmonary arteries in patients with pulmonary atresia and ventricular septal defect (VSD) beyond the infant period. Methods: From December 2009 to August 2012, 37 consecutive patients (mean age 1.88 ± 1.67 [range 0.6-7.2] years) diagnosed with pulmonary atresia, VSD, pulmonary artery hypoplasia (Nakata index 90.92 ± 42.6 mm2/m2, McGoon 1.02 ± 0.22) were included in this retrospective study. All patients underwent the procedure of right ventricle to pulmonary artery connection, most with concomitant major aortopulmonary collateral transcatheter occlusion and/or pulmonary angioplasty. Mean follow-up was 1.59 ± 0.76 years. Results: There was no early death but one patient died of myocarditis one year after the rehabilitation. Significant pulmonary artery growth was obtained (Nakata 215.07 ± 95.05 mm2/m2, McGoon 1.64 ± 0.52, P < 0.001). Seventeen patients (54.8%) whose pulmonary growth was considered adequate obtained complete repair with VSD closure. The preoperative McGoon index is a good predictor for adequate pulmonary growth. There was one early death after anatomical repair. At last follow-up, all the survivors who underwent anatomical repair were in New York Heart Association class I-II with satisfactory haemodynamics. Conclusions: Right ventricle to pulmonary artery connection is safe and effective in promoting the growth of the native pulmonary arteries in patients with pulmonary atresia, ventricular septal defect and hypoplastic native pulmonary arteries. Ultimately, this strategy allows complete repair in the majority of patients beyond the infant period.
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