Long-term results of pulmonary artery rehabilitation in patients with pulmonary atresia, ventricular septal defect, pulmonary artery hypoplasia, and major aortopulmonary collaterals

ObjectiveThe study objective was to report the long-term results of pulmonary artery rehabilitation in patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries, and major aortopulmonary collaterals.MethodsSince 1991, 20 patients with profound pulmonary artery hypoplasia (mean Nakata index 26 ± 14 mm2/m2) have undergone a medico-surgical strategy of native pulmonary artery rehabilitation to achieve complete repair with satisfactory hemodynamics (right ventricle to aortic pressure ratio < 0.8).ResultsThe first step, right ventricle to pulmonary artery connection, was performed at a median age of 4.1 (0.1–18.7) months with 1 operative death. After a median duration of 4.3 (1.1–26) months, the second step of interventional catheterizations followed (median, 2 (1–7)/patient), consisting of 36 pulmonary angioplasties, 11 stent implantations, and 20 collateral occlusions. Significant pulmonary artery growth was obtained in all cases with a Nakata index of 208 ± 85 mm2/m2 before surgical correction (P < .001). The third step of surgical repair was performed at a median age of 1.9 (0.6–10.7) years, with right ventricular outflow reconstruction and ventricular septal defect closure fenestrated in 3 cases. During a mean follow-up of 8.2 ± 4.5 years, pulmonary artery rehabilitation was pursued in most patients, with 47 pulmonary angioplasties, 15 stent implantations, and 11 collateral occlusions. Three patients with a poor hemodynamic result died. At last visit, the 16 survivors are in New York Heart Association class I (n = 12) or II (n = 4) with satisfactory hemodynamics in 13 cases.ConclusionsPulmonary artery rehabilitation allows complete repair in the majority of patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries, and major aortopulmonary collaterals. However, long-term management often requires pursuit of the rehabilitation process