Transplantation-Free Survival and Interventions at 3 Years in the Single Ventricle Reconstruction Trial

• Type of Material: Article
• Authors: Jane W. Newburger, Boston Children's Hospital; Lynn A. Sleeper, New England Research Institutes; J. William Gaynor, Childrens Hospital of Philadelphia; Danielle Hollenbeck-Pringle, New England Research Institutes; Peter C. Frommelt, Childrens Hospital of Wisconsin; Jennifer S. Li, North Carolina Consortium; William Mahle, Emory University; Ismee A. Williams, Morgan Stanley Children's Hospital; Andrew M. Atz, Medical University of South Carolina; Kristin M. Burns, National Institutes of Health; Shan Chen, New England Research Institutes; James Cnota, Cincinnati Children's Medical Center; Carolyn Dunbar-Masterson, Boston Children's Hospital; Nancy S. Ghanayem, Medical College of Wisconsin; Caren S. Goldberg, University of Michigan; Jeffrey P. Jacobs, Congenital Heart Institute of Florida; Alan B. Lewis, Childrens Hospital Los Angeles; Seema Mital, Hospital for Sick Children; Christian Pizarro, Nemours Cardiac Center; Aaron Eckhauser, University of Utah; Paul Stark, New England Research Institutes; Richard G. Ohye, University of Michigan
• Language: English
• Date: 2014-05-20
• Publisher: American Heart Association
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2014 American Heart Association, Inc.
• Final Published Version (URL): http://dx.doi.org/10.1161/CIRCULATIONAHA.113.006191
• Title of Journal or Parent Work: Circulation
• ISSN: 0009-7322
• Volume: 129
• Issue: 20
• Start Page: 2013
• End Page: +
• Grant/Funding Information: This work was supported by grants (HL068269, HL068270, HL068279, HL068281, HL068285, HL068288, HL068290, HL068292, and HL085057) from the National Heart, Lung, and Blood Institute (NHLBI).
• Keywords: heart defects, congenital; Peripheral Vascular Disease; Life Sciences & Biomedicine; FONTAN; OPERATION; AORTOPULMONARY COLLATERAL VESSELS; PULMONARY-ARTERY CONDUIT; Norwood procedures; Science & Technology; Cardiac & Cardiovascular Systems; cardiac surgical procedures; heart ventricles; heart diseases; BLALOCK-TAUSSIG SHUNT; RISK-FACTORS; Cardiovascular System & Cardiology; LEFT-HEART SYNDROME; QUANTIFICATION; NORWOOD PROCEDURE; MORTALITY
• Research Categories: Health Sciences, Medicine and Surgery

Abstract

BACKGROUND - : In the Single Ventricle Reconstruction (SVR) trial, 1-year transplantation-free survival was better for the Norwood procedure with right ventricle-to-pulmonary artery shunt (RVPAS) compared with a modified Blalock-Taussig shunt (MBTS). At 3 years, we compared transplantation-free survival, echocardiographic right ventricular ejection fraction, and unplanned interventions in the treatment groups. METHODS AND RESULTS - : Vital status and medical history were ascertained from annual medical records, death indexes, and phone interviews. The cohort included 549 patients randomized and treated in the SVR trial. Transplantation-free survival for the RVPAS versus MBTS groups did not differ at 3 years (67% versus 61%; P=0.15) or with all available follow-up of 4.8±1.1 years (log-rank P=0.14). Pre-Fontan right ventricular ejection fraction was lower in the RVPAS group than in the MBTS group (41.7±5.1% versus 44.7±6.0%; P=0.007), and right ventricular ejection fraction deteriorated in RVPAS (P=0.004) but not MBTS (P=0.40) subjects (pre-Fontan minus 14-month mean, -3.25±8.24% versus 0.99±8.80%; P=0.009). The RVPAS versus MBTS treatment effect had nonproportional hazards (P=0.004); the hazard ratio favored the RVPAS before 5 months (hazard ratio=0.63; 95% confidence interval, 0.45-0.88) but the MBTS beyond 1 year (hazard ratio=2.22; 95% confidence interval, 1.07-4.62). By 3 years, RVPAS subjects had a higher incidence of catheter interventions (P<0.001) with an increasing HR over time (P=0.005): <5 months, 1.14 (95% confidence interval, 0.81-1.60); from 5 months to 1 year, 1.94 (95% confidence interval, 1.02-3.69); and >1 year, 2.48 (95% confidence interval, 1.28-4.80). CONCLUSIONS - : By 3 years, the Norwood procedure with RVPAS compared with MBTS was no longer associated with superior transplantation-free survival. Moreover, RVPAS subjects had slightly worse right ventricular ejection fraction and underwent more catheter interventions with increasing hazard ratio over time.