Transcatheter pulmonary valvuloplasty in neonates with pulmonary atresia and intact ventricular septum

Summary Background Transcatheter pulmonary valvuloplasty in neonates with pulmonary atresia and intact ventricular septum (PA-IVS) or duct-dependent pulmonary valve stenosis (DD-PVS) has become a reasonable alternative to surgical right ventricle decompression. Aim To investigate mid-term outcomes following pulmonary valvuloplasty. Methods Sixty-five neonates with PA-IVS ( n  = 29) or DD-PVS ( n  = 36) (median age 4 days; mean weight 3.0 kg) undergoing pulmonary valvuloplasty were reviewed retrospectively. Procedural data and clinical outcomes were assessed. Results Pulmonary valvuloplasty was successful in 59 patients (90.8%). Preterm birth, larger tricuspid valve annulus diameter and PA-IVS correlated with procedural failure. Eleven patients (18.6%) required a Blalock-Taussig shunt during early follow-up, despite valvuloplasty. These neonates had smaller tricuspid and pulmonary valve annulus Z-scores (–1.9 vs. –0.8 [ p  = 0.04] and –2.5 vs. –0.9 [ P  = 0.005], respectively) and a higher incidence of “bipartite” right ventricle ( P  = 0.02). Mean follow-up was 5.4 ± 3.3 years. Mortality after successful valvuloplasty was 8.5% ( n  = 5). Among the 54 survivors, biventricular repair was achieved in 52 patients (96.3%), including nine with a previous Blalock-Taussig shunt. The cumulative rate of subsequent surgery (excluding Blalock-Taussig shunt) was 13.7% (95% confidence interval 6.8–26.7%) and 16.4% (95% confidence interval 8.5–30.4%) at 2 and 4 years, respectively. Secondary surgery was significantly more frequent in PA-IVS compared with DD-PVS, and in neonates with a Blalock-Taussig shunt ( P  = 0.003 and 0.01, respectively). Conclusions Selected neonates with DD-PVS or PA-IVS managed by transcatheter pulmonary valvuloplasty had a good mid-term outcome. In neonates with a borderline small right ventricle, a hybrid strategy with a supplementary source of pulmonary blood flow can be efficient to achieve biventricular repair.