Transplant-Free Survival and Interventions at 6 Years in the SVR Trial

• Type of Material: Article
• Authors: Jane W. Newburger, Boston Children’s Hospital; Lynn A. Sleeper, Boston Children’s Hospital; J. William Gaynor, Children's Hospital of Philadelphia; Danielle Hollenbeck-Pringle, New England Research Institutes; Peter C. Frommelt, Children’s Hospital of Wisconsin; Jennifer S. Li, Duke University; William Mahle, Emory University; Ismee A. Williams, Morgan Stanley Children’s Hospital of New York-Presbyterian; Andrew M. Atz, Medical University of South Carolina; Kristin M. Burns, National Heart, Lung, and Blood Institute; Shan Chen, New England Research Institutes; James Cnota, Cincinnati Children’s Medical Center; Carolyn Dunbar-Masterson, Boston Children's Hospital; Nancy S. Ghanayem, Children’s Hospital of Wisconsin; Caren S. Goldberg, University of Michigan; Jeffrey P. Jacobs, Congenital Heart Institute of Florida; Alan B. Lewis, Children's Hospital Los Angeles; Seema Mital, Hospital for Sick Children; Christian Pizarro, Nemours Cardiac Center; Aaron Eckhauser, Primary Children’s Hospital; Paul Stark, New England Research Institutes; Richard G. Ohye, University of Michigan
• Language: English
• Date: 2018-05-22
• Publisher: American Heart Association
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2018 American Heart Association, Inc.
• Final Published Version (URL): http://dx.doi.org/10.1161/CIRCULATIONAHA.117.029375
• Title of Journal or Parent Work: Circulation
• ISSN: 0009-7322
• Volume: 137
• Issue: 21
• Start Page: 2246
• End Page: 2253
• 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: SHUNT; single ventricle; Life Sciences & Biomedicine; congenital heart defect; Cardiac & Cardiovascular Systems; congenital heart disease; LEFT-HEART SYNDROME; ARRHYTHMIA; cardiac surgery; NORWOOD PROCEDURE; Norwood procedure; Cardiovascular System & Cardiology; FONTAN OPERATION; Science & Technology; DEATH; VENTRICLE RECONSTRUCTION TRIAL; LESIONS; RISK; Peripheral Vascular Disease
• Research Categories: Health Sciences, Medicine and Surgery

Abstract

BACKGROUND: In the SVR trial (Single Ventricle Reconstruction), 1-year transplant-free survival was better for the Norwood procedure with right ventricle-to-pulmonary artery shunt (RVPAS) compared with a modified Blalock‒Taussig shunt in patients with hypoplastic left heart and related syndromes. At 6 years, we compared transplant-free survival and other outcomes between the groups. METHODS: Medical history was collected annually using medical record review, telephone interviews, and the death index. The cohort included 549 patients randomized and treated in the SVR trial. RESULTS: Transplant-free survival for the RVPAS versus modified Blalock‒Taussig shunt groups did not differ at 6 years (64% versus 59%, P=0.25) or with all available follow-up of 7.1±1.6 years (log-rank P=0.13). The RVPAS versus modified Blalock‒Taussig shunt treatment effect had nonproportional hazards (P=0.009); the hazard ratio (HR) for death or transplant favored the RVPAS before stage II surgery (HR, 0.66; 95% confidence interval, 0.48-0.92). The effect of shunt type on death or transplant was not statistically significant between stage II to Fontan surgery (HR, 1.36; 95% confidence interval, 0.86-2.17; P=0.17) or after the Fontan procedure (HR, 0.76; 95% confidence interval, 0.33-1.74; P=0.52). By 6 years, patients with RVPAS had a higher incidence of catheter interventions (0.38 versus 0.23/patient-year, P<0.001), primarily because of more interventions between the stage II and Fontan procedures (HR, 1.72; 95% confidence interval, 1.00-3.03). Complications did not differ by shunt type; by 6 years, 1 in 5 patients had had a thrombotic event, and 1 in 6 had had seizures. CONCLUSIONS: By 6 years, the hazards of death or transplant and catheter interventions were not different between the RVPAS versus modified Blalock‒Taussig shunt groups. Children assigned to the RVPAS group had 5% higher transplant-free survival, but the difference did not reach statistical significance, and they required more catheter interventions. Both treatment groups have accrued important complications.