Long-term outcomes after intervention for pulmonary atresia with intact ventricular septum

• Type of Material: Article
• Authors: Lydia K. Wright, Emory University; Jessica H. Knight, University of Georgia; Amanda S. Thomas, Emory University; Matthew Oster, Emory University; James D. St Louis, University of Missouri; Lazaros Kochilas, Emory University
• Language: English
• Date: 2019-07-01
• Publisher: BMJ Publishing Group
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.
• Final Published Version (URL): http://dx.doi.org/10.1136/heartjnl-2018-314124
• Title of Journal or Parent Work: Heart
• Volume: 105
• Issue: 13
• Start Page: 1007
• End Page: 1013
• Grant/Funding Information: This study was supported by the National Heart, Lung, and Blood institute (R01 HL122392).
• Keywords: Cardiac & Cardiovascular Systems; Mortality; pulmonary atresia; long-term outcomes; Coronary arterial abnormalities; congenital heart surgery; Cardiovascular System & Cardiology; congenital heart defect; Circulation; Life Sciences & Biomedicine; Repair; Management; Predictors; Science & Technology
• Research Categories: Health Sciences, Medicine and Surgery

Abstract

Objective Pulmonary atresia with intact ventricular septum (PA/IVS) can be treated by various operative and catheter-based interventions. We aim to understand the long-term transplant-free survival of patients with PA/IVS by treatment strategy. Methods Cohort study from the Pediatric Cardiac Care Consortium, a multi-institutional registry with prospectively acquired outcome data after linkage with the National Death index and the Organ Procurement and Transplantation Network. Results Eligible patients underwent neonatal surgery or catheter-based intervention for PA/IVS between 1982 and 2003 (median follow-up of 16.7 years, IQR: 12.6–22.7). Over the study period, 616 patients with PA/IVS underwent one of three initial interventions: aortopulmonary shunt, right ventricular decompression or both. Risk factors for death at initial intervention included earlier birth era (1982–1992), chromosomal abnormality and atresia of one or both coronary ostia. Among survivors of neonatal hospitalisation (n=491), there were 99 deaths (4 post-transplant) and 10 transplants (median age of death or transplant 0.7 years, IQR: 0.3–1.8 years). Definite repair or last-stage palliation was achieved in the form of completed two-ventricle repair (n=201), one-and-a-half ventricle (n=39) or Fontan (n=96). Overall 20-year survival was 66%, but for patients discharged alive after definitive repair, it reached 97.6% for single-ventricle patients, 90.9% for those with one-and-a-half ventricle and 98.0% for those with complete two-ventricle repair (log-rank p=0.052). Conclusions Transplant-free survival in PA/IVS is poor due to significant infantile and interstage mortality. Survival into early adulthood is excellent for patients reaching completion of their intended path independent of type of repair.