Outcomes of transcatheter approach for initial treatment of pulmonary atresia with intact ventricular septum

Abstract

To review the short and medium term outcome of transcatheter pulmonary valve perforation (PVP) in patients with pulmonary atresia-intact ventricular septum and non-right ventricular dependant coronary circulation (PA/IVS non-RVDCC). PVP in patients with PA/IVS non-RVDCC has become more common in the past two decades. However, data on outcomes with this strategy are mixed. Data were reviewed retrospectively for all patients with PA/IVS non-RVDCC treated from 1996- 2010 at our institution. Patients who had severe neonatal Ebstein malformation, or initial interventional management at another institution were excluded. PVP was attempted in 30 of 50 patients (60%); 26 (87%) of these had a successful procedure. Twenty-four patients (48%) had surgery without PVP. There were no deaths in the cohort. Complications of PVP included 5 (17%) myocardial perforations. Of those with successful PVP, 10 (38%) did not have surgery (PVP-NS) and 16 (62%) had surgery (PVP-S) prior to discharge. Tricuspid valve (TV) Z-score was larger in the PVP-NS than in PVP-S patients, with median TV diameter Z-scores of +0.7 (-0.9, 1.7) and -1.1 (-2.8, 2), respectively (P = 0.01). Time from PVP to either hospital discharge (PVP-NS group) or surgery (PVP-S group) was significantly different between groups: 15 (7, 22) and 8 days (0, 46), respectively (P = 0.01). There were no differences in the number of trials or lowest arterial PaO2 off prostaglandins between groups. All patients in the PVP-NS group had a biventricular circulation at a median follow-up of 4.3 years. The results of a collaborative approach to treating neonates with PA/IVS non-RVDCC are excellent. Smaller TV size is associated with greater likelihood of surgery prior to discharge, and may serve as a surrogate for early RV inadequacy.