Transplantation-free survival and interventions at 3 years in the single ventricle reconstruction trial

Jane W Newburger; Lynn A Sleeper; Peter C Frommelt; Gail D Pearson; William T Mahle; Shan Chen; Carolyn Dunbar-Masterson; Seema Mital; Ismee A Williams; Nancy S Ghanayem; Caren S Goldberg; Jeffrey P Jacobs; Catherine D Krawczeski; Alan B Lewis; Sara K Pasquali; Christian Pizarro; Peter J Gruber; Andrew M Atz; Svetlana Khaikin; J William Gaynor; Richard G Ohye; Pediatric Heart Network Investigators

doi:10.1161/CIRCULATIONAHA.113.006191

Transplantation-free survival and interventions at 3 years in the single ventricle reconstruction trial

Circulation. 2014 May 20;129(20):2013-20. doi: 10.1161/CIRCULATIONAHA.113.006191. Epub 2014 Apr 4.

Authors

Jane W Newburger¹, Lynn A Sleeper², Peter C Frommelt², Gail D Pearson², William T Mahle², Shan Chen², Carolyn Dunbar-Masterson², Seema Mital², Ismee A Williams², Nancy S Ghanayem², Caren S Goldberg², Jeffrey P Jacobs², Catherine D Krawczeski², Alan B Lewis², Sara K Pasquali², Christian Pizarro², Peter J Gruber², Andrew M Atz², Svetlana Khaikin², J William Gaynor², Richard G Ohye²; Pediatric Heart Network Investigators

Affiliations

¹ From the Boston Children's Hospital and Harvard Medical School, Boston, MA (J.W.N., C.D.-M.); New England Research Institutes, Watertown, MA (L.A.S., S.C.); Children's Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee (P.C.F.); National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD (G.D.P.); Emory University, Atlanta, GA (W.T.M.); Hospital for Sick Children, Toronto, ON, Canada (S.M., S.K.); Morgan Stanley Children's Hospital of New York-Presbyterian, New York (I.A.W.); University of Michigan Medical School, Ann Arbor (C.S.G., R.G.O.); The Congenital Heart Institute of Florida, St. Petersburg (J.P.J.); Cincinnati Children's Medical Center, Cincinnati, OH (C.D.K.); Children's Hospital Los Angeles, Los Angeles, CA (A.B.L.); North Carolina Consortium: Duke University, Durham; East Carolina University, Greenville; Wake Forest University, Winston-Salem (S.K.P.); Nemours Cardiac Center, Wilmington, DE (C.P.); Primary Children's Medical Center and University of Utah, Salt Lake City (P.J.G.); Medical University of South Carolina, Charleston (A.M.A.); and Children's Hospital of Philadelphia and University of Pennsylvania Medical School, Philadelphia (J.W.G.). jane.newburger@cardio.chboston.org.
² From the Boston Children's Hospital and Harvard Medical School, Boston, MA (J.W.N., C.D.-M.); New England Research Institutes, Watertown, MA (L.A.S., S.C.); Children's Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee (P.C.F.); National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD (G.D.P.); Emory University, Atlanta, GA (W.T.M.); Hospital for Sick Children, Toronto, ON, Canada (S.M., S.K.); Morgan Stanley Children's Hospital of New York-Presbyterian, New York (I.A.W.); University of Michigan Medical School, Ann Arbor (C.S.G., R.G.O.); The Congenital Heart Institute of Florida, St. Petersburg (J.P.J.); Cincinnati Children's Medical Center, Cincinnati, OH (C.D.K.); Children's Hospital Los Angeles, Los Angeles, CA (A.B.L.); North Carolina Consortium: Duke University, Durham; East Carolina University, Greenville; Wake Forest University, Winston-Salem (S.K.P.); Nemours Cardiac Center, Wilmington, DE (C.P.); Primary Children's Medical Center and University of Utah, Salt Lake City (P.J.G.); Medical University of South Carolina, Charleston (A.M.A.); and Children's Hospital of Philadelphia and University of Pennsylvania Medical School, Philadelphia (J.W.G.).

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.

Clinical trial registration url: http://www.clinicaltrials.gov. Unique identifier: NCT00115934.

Keywords: Norwood procedures; cardiac surgical procedures; heart defects, congenital; heart diseases; heart ventricles.

Publication types

Comparative Study
Multicenter Study
Randomized Controlled Trial
Research Support, N.I.H., Extramural

MeSH terms

Aorta, Thoracic / surgery
Blalock-Taussig Procedure / methods
Blalock-Taussig Procedure / mortality*
Cardiac Catheterization / statistics & numerical data
Child, Preschool
Disease-Free Survival
Female
Follow-Up Studies
Heart Transplantation
Heart Ventricles / abnormalities*
Heart Ventricles / surgery
Humans
Hypoplastic Left Heart Syndrome / mortality*
Hypoplastic Left Heart Syndrome / surgery*
Incidence
Infant
Logistic Models
Male
Norwood Procedures / methods
Norwood Procedures / mortality*
Proportional Hazards Models
Prospective Studies
Pulmonary Artery / surgery
Risk Factors
Treatment Outcome
Ventricular Function, Right

Associated data

ClinicalTrials.gov/NCT00115934

Abstract

Publication types

MeSH terms

Associated data

Grants and funding