Author + information
- Christine Bui,
- Wilson Lam,
- Wayne Franklin and
- Peter Ermis
Background: Early childhood palliation for pulmonary atresia with intact ventricular septum (PA-IVS) can range from biventricular repair to single ventricle palliation. This study aims to evaluate the need for additional interventions long-term in a survivor cohort of adult PA-IVS patients following initial surgical palliation.
Methods: All patients 18 years of age and older with a diagnosis of PA-IVS seen in the adult congenital cardiology clinic at our institution from 2010 – 2016 were included. Charts were retrospectively reviewed for initial surgical repair, demographics, and long-term co-morbidities.
Results: Thirty-seven PA-IVS adults were identified. The mean age was 24.4 (18-43) years. Initial surgical repair was a Fontan operation (18, 49%), a 1.5 ventricular repair (5, 14%), or a biventricular repair (14, 38%). Mean follow-up after initial palliation was 18.9 (12-36) years. There were no deaths within the study period (2010-2016). One patient required cardiac transplantation 13 years after initial palliation. Seventeen patients (46%) required re-intervention at an average of 13.3 (3-34) years following initial palliation. Four (22%) Fontan patients required a Fontan revision. Three (60%) patients with 1.5 ventricular repair required conduit replacement, stent implantation, or angioplasty. Ten (71%) biventricular patients required re-intervention; pulmonary valve replacement was the most common (n=8, one percutaneous), followed by tricuspid valve repair or replacement (n=4, one percutaneous). Atrial arrhythmias were present in 13 (35%) and ventricular arrhythmias in 9 (24%) patients. Three patients had clinically significant liver disease.
Conclusions: We report the largest cohort of adult survivors with palliated PA-IVS following initial palliation. During a limited study interval, there was no mortality. While surgical or catheter-based re-interventions were often performed, the need for re-intervention in this group was much less than previously reported (46% vs 100%). Additional studies are needed that could illustrate the optimal timing and indication for re-intervention in PA-IVS patients.
Moderated Poster Contributions
Congenital Heart Disease and Pulmonary Hypertension Moderated Poster Theater, Poster Hall, Hall C
Friday, March 17, 2017, 11:45 a.m.-11:55 a.m.
Session Title: Congenital Heart Disease: To the Third Decade and Beyond
Abstract Category: 9. Congenital Heart Disease: Adult
Presentation Number: 1131M-17
- 2017 American College of Cardiology Foundation