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

Abstract

Objective: The 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.

Methods: Since 1991, 20 patients with profound pulmonary artery hypoplasia (mean Nakata index 26 ± 14 mm(2)/m(2)) 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).

Results: The 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 mm(2)/m(2) 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.

Conclusions: Pulmonary 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.