Hybrid approach for pulmonary atresia with intact ventricular septum: early single center results and comparison to the standard surgical approach.

OBJECTIVES: To examine acute and mid-term patient outcomes following the hybrid approach to pulmonary atresia with intact ventricular septum (PA-IVS) compared with the standard surgical approach. BACKGROUND: A subset of PA-IVS patients with the prospect of biventricular circulation typically undergo surgical or transcatheter right ventricular (RV) outflow tract opening. A recently described hybrid procedure, involving perventricular pulmonary valve perforation, was shown to be safe and effective in single-center series. METHODS: A single-center retrospective review of all patient with PA-IVS who underwent either surgical or hybrid RV decompression between January 2002 and December 2011 was completed and acute and mid-term patient outcomes were compared between the surgical and hybrid cohorts. Additionally, a systematic literature review was completed to compare a transcatheter cohort to the hybrid cohort. RESULTS: Seven patients with PA-IVS underwent a hybrid procedure; the procedure was technically successful in all attempts, and none required CPB. No patients required surgical re-intervention prior to hospital discharge, and none died during the study period. Surgical RV decompression was performed in 17 patients with a median CPB time of 80 min. Patient outcomes were nearly identical between cohorts. By systematic review, the transcatheter approach has a procedural success of 75-95% but up to 75% of patients require operation in the neonatal period. CONCLUSIONS: The hybrid approach is a safe and feasible alternative to the standard surgical and transcatheter approaches to PA-IVS. Acute and mid-term patient outcomes are comparable with those treated with a standard surgical approach and neonatal CPB is completely avoided.