Concomitant stenting of the patent ductus arteriosus and radiofrequency valvotomy in pulmonary atresia with intact ventricular septum and intermediate right ventricle: early in-hospital and medium-term outcomes.

OBJECTIVES: Our objective was to determine the feasibility and early to medium-term outcome of stenting the patent ductus arteriosus at the time of radiofrequency valvotomy in the subgroup of patients with pulmonary atresia with intact ventricular septum and intermediate right ventricle. BACKGROUND: Stenting of the patent ductus arteriosus and radiofrequency valvotomy have been proposed as the initial intervention for patients with intermediate right ventricle inasmuch as the sustainability for biventricular circulation or 1½-ventricle repair is unclear in the early period. METHODS: Between January 2001 and April 2009, of 143 patients with pulmonary atresia and intact ventricular septum, 37 who had bipartite right ventricle underwent radiofrequency valvotomy and stenting of the patent ductus arteriosus as the initial procedure. The mean tricuspid valve z-score was -3.8 ± 2.2 and the mean tricuspid valve/mitral valve ratio was 0.62 ± 0.16. RESULTS: Median age was 10 days (3-65 days) and median weight 3.1 kg (2.4-4.9 kg). There was no procedural mortality. Acute stent thrombosis developed in 1 patient and necessitated emergency systemic-pulmonary shunt. There were 2 early in-hospital deaths owing to low cardiac output syndrome. One late death occurred owing to right ventricular failure after the operation. Survival after the initial procedure was 94% at 6 months and 91% at 5 years. At a median follow-up of 4 years (6 months to 8 years), 17 (48%) attained biventricular circulation with or without other interventions and 9 (26%) achieved 1½-ventricle repair. The freedom from reintervention was 80%, 68%, 58%, and 40% at 1, 2, 3, and 4 years, respectively. CONCLUSIONS: Concomitant stenting of the patent ductus arteriosus at the time of radiofrequency valvotomy in patients with pulmonary atresia with intact ventricular septum and intermediate right ventricle is feasible and safe with encouraging medium-term outcome.