Objectives. To report long-term results of pulmonary artery rehabilitation in pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries and major aortopulmonary collaterals.
Methods. Since 1993, 20 patients with profound pulmonary arteries hypoplasia (mean Nakata index 26±14mm²/m²) benefited from a medico-surgical strategy of native pulmonary arteries rehabilitation with an ultimate goal to achieve anatomical 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 one operative death. After a median duration of 4.3(1.1-26)months, followed the 2nd step of interventional catheterizations (median: 2(1-7)/patient) consisting of 36 pulmonary angioplasties, 11 stent implantation and 20 collaterals occlusions. Significant pulmonary arteries growth was obtained in all cases with a Nakata index of 208±85mm²/m² before surgical correction (p<0.001). The 3rd 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 patch closure, fenestrated in 3 cases. During a mean follow-up of 8.2±4.5 years, pulmonary arteries rehabilitation was pursued in most patients, with 47 pulmonary angioplasties, 15 stent implantation and 11 collaterals occlusion. Three patients with poor hemodynamic result died. At last visit, the 16 survivors are in NYHA class I (n= 12) or II (n= 4) with satisfactory hemodynamics in 13 cases.
Conclusion. Medico-surgical pulmonary arteries rehabilitation is successful in the majority of cases allowing complete repair with an early and aggressive approach. However, long term management often requires pursuit of the rehabilitation process.