Long-Term Outcome Following Catheter Valvotomy for Pulmonary Atresia With Intact Ventricular Septum

Study Questions:

What are the long-term outcomes for patients undergoing catheter perforation for pulmonary atresia with intact ventricular septum?

Methods:

A retrospective review was performed at a single center. Patients with right ventricle-dependent coronary circulation were excluded. A total of 39 patients were referred for catheter valve perforation during the course of the study period. Perforation was performed with a laser for five of the early patients; the remainder underwent radiofrequency valve perforation.

Results:

Valve perforation was successful in 37 of 39 patients. Seventeen patients also underwent stenting of the patent ductus arteriosus. The mean (standard deviation) tricuspid valve z-score was -5.1 (± 3.4). Eight deaths (21%) were reported, all of which occurred within the first 35 days. The vast majority (83%) of survivors have a biventricular circulation. Stenting of the patent ductus arteriosus was associated with significant reductions in early re-intervention, as well as hospital stay.

Conclusions:

Long-term survival after catheter perforation is good, and even patients with small tricuspid valves and right ventricles may be candidates for the procedure. Stenting of the patent ductus arteriosus may reduce hospital stay and repeat procedures.

Perspective:

The study demonstrates excellent long-term results for catheter perforation of the pulmonary valve in patients with pulmonary atresia with an intact ventricular septum. This is despite the inclusion of patients with small right ventricles and tricuspid valves (mean tricuspid valve z-score of -5.1). The investigators note the value of stenting of the patent ductus arteriosus in reducing hospital stay and the need for additional neonatal interventions. The primary limitation of the study is the fact that it is a single-center study at a highly experienced center. Results will need to be confirmed at additional centers.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Valvular Heart Disease, Congenital Heart Disease, CHD & Pediatrics and Interventions, Interventions and Structural Heart Disease

Keywords: Tricuspid Valve, Heart Defects, Congenital, Lasers, Pulmonary Atresia, Coronary Circulation, Catheters, Pulmonary Valve, Heart Ventricles, Ductus Arteriosus, Patent, Stents, Radio Waves


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