Multistage pulmonary artery rehabilitation in patients with pulmonary atresia, ventricular septal defect and hypoplastic pulmonary artery

Abstract

Objectives: The aim of this study was to determine the effect of multistage pulmonary artery (PA) rehabilitation consisting of right ventricle to pulmonary artery (RV-PA) connection, major aortopulmonary collateral artery (MAPCA) closure and PA angioplasty in patients with pulmonary atresia, ventricular septal defect (VSD) and hypoplastic pulmonary arteries. In addition, the effects of the PA reintervention were reported and risk factors were analysed.

Methods: This study was a retrospective review of 69 consecutive patients with pulmonary atresia, VSD and hypoplastic pulmonary arteries (mean Nakata index 100.9 ± 57.6 mm(2)/m(2)) who underwent multistage rehabilitation of hypoplastic PA from December 2009 to December 2014.

Results: RV-PA connection was performed at a median age of 1.0 years with 2 hospital deaths in the hybrid operation theatre. Thirty-two patients underwent concomitant pulmonary angioplasty with 28 collateral occlusions. After a mean duration of 15.4 ± 12.7 months, 16 patients had interventional catheterization consisting of 14 balloon dilatations, 12 stent implantations and 16 collateral occlusions. At a mean age of 2.7 ± 1.9 years, complete repair was performed in 31 patients within 1.2 ± 0.6 years of the palliation with 1 hospital death. Twenty-two patients had concomitant PA angioplasty. The estimated complete repair rate was 60.1 ± 7.1% ∼3 years after the palliation by the Kaplan-Meier method. During a mean follow-up of 2.8 ± 1.3 years, 49.3% (33/67) of the patients had PA reintervention. Pulmonary stenosis requiring angioplasty at palliation is associated with PA reintervention (P = 0.003). The actuarial survival rate for the overall population was 93.8 ± 3.0% at 5 years after the placement of an RV-PA connection.

Conclusions: The multistage strategy consisting of a RV-PA connection, MAPCA closure and PA angioplasty is effective in rehabilitating the hypoplastic PA in patients with pulmonary atresia, VSD and hypoplastic pulmonary arteries. However, PA reinterventions may be required in specific patients.

Keywords: Pulmonary arteries; Pulmonary atresia; Rehabilitation; Right ventricle to pulmonary artery connection.