Longitudinal Follow-Up of Children With HLHS and Association Between Norwood Shunt Type and Long-Term Outcomes: The SVR III Study-Reference-Cited by-同舟云学术

Longitudinal Follow-Up of Children With HLHS and Association Between Norwood Shunt Type and Long-Term Outcomes: The SVR III Study

Published:2023-10-24 Issue:17 Volume:148 Page:1330-1339
ISSN:0009-7322
Container-title:Circulation
language:en
Short-container-title:Circulation

Author:

Goldberg Caren S.¹^ORCID,Trachtenberg Felicia²,William Gaynor J.³⁴,Mahle William T.⁵,Ravishankar Chitra⁶⁷^ORCID,Schwartz Steven M.⁸^ORCID,Cnota James F.⁹^ORCID,Ohye Richard G.¹⁰,Gongwer Russell²^ORCID,Taylor Michael¹¹^ORCID,Paridon Stephen⁶⁷^ORCID,Frommelt Peter C.¹²^ORCID,Afton Katherine¹³^ORCID,Atz Andrew M.¹⁴^ORCID,Burns Kristin M.¹⁵^ORCID,Detterich Jon A.¹⁶^ORCID,Hill Kevin D.¹⁷^ORCID,Cabrera Antonio G.¹⁸,Lewis Alan B.¹⁶^ORCID,Pizarro Christian¹⁹^ORCID,Shah Amee²⁰,Sharma Binu²,Newburger Jane W.²¹^ORCID,

Affiliation:

1. C.S. Mott Children’s Hospital (C.S.G.), University of Michigan, Ann Arbor.

2. Carelon Research, Newton, MA (F.T., R.G., B.S.).

3. Departments of Surgery (J.W.G.), Children’s Hospital of Philadelphia, PA.

4. Departments of Surgery (J.W.G.), Perelman School of Medicine, University of Pennsylvania, Philadelphia.

5. Department of Pediatrics, Children’s Healthcare of Atlanta, GA (W.T.M.).

6. Pediatrics (C.R., S.P.), Children’s Hospital of Philadelphia, PA.

7. Pediatrics (C.R., S.P.), Perelman School of Medicine, University of Pennsylvania, Philadelphia.

8. Department of Critical Care Medicine, the Hospital for Sick Children, Toronto, Ontario, Canada (S.M.S.).

9. Division of Pediatric Cardiology, Cincinnati Children’s Hospital, OH (J.F.C.).

10. Department of Cardiac Surgery (R.G.O.), University of Michigan, Ann Arbor.

11. Department of Pediatrics, Cincinnati Children’s Hospital and Medical Center, OH (M.T.).

12. Department of Pediatrics, Children’s Wisconsin and the Medical College of Wisconsin, Milwaukee (P.C.F.).

13. Michigan Congenital Heart Center Research and Discovery (K.A.), University of Michigan, Ann Arbor.

14. Department of Pediatrics, Medical University of South Carolina, Charleston (A.A.).

15. National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD (K.M.B.).

16. Department of Pediatrics, Children’s Hospital, Los Angeles, CA (J.A.D., A.B.L.).

17. Department of Pediatrics, Duke University, Durham, NC (K.D.H.).

18. Department of Pediatrics, University of Utah, Salt Lake City (A.G.C.).

19. Nemours Cardiac Center, Department of Cardiovascular Medicine, Nemours Children’s Health, Wilmington, DE (C.P.).

20. Department of Pediatrics, Columbia University Medical Center, New York, NY (A.S.).

21. Department of Pediatric Cardiology, Boston Children’s Hospital, MA (J.W.N.).

Abstract

OBJECTIVE: In the SVR trial (Single Ventricle Reconstruction), newborns with hypoplastic left heart syndrome were randomly assigned to receive a modified Blalock-Taussig-Thomas shunt (mBTTS) or a right ventricle-to-pulmonary artery shunt (RVPAS) at Norwood operation. Transplant-free survival was superior in the RVPAS group at 1 year, but no longer differed by treatment group at 6 years; both treatment groups had accumulated important morbidities. In the third follow-up of this cohort (SVRIII [Long-Term Outcomes of Children With Hypoplastic Left Heart Syndrome and the Impact of Norwood Shunt Type]), we measured longitudinal outcomes and their risk factors through 12 years of age. METHODS: Annual medical history was collected through record review and telephone interviews. Cardiac magnetic resonance imaging (CMR), echocardiogram, and cycle ergometry cardiopulmonary exercise tests were performed at 10 through 14 years of age among participants with Fontan physiology. Differences in transplant-free survival and complication rates (eg, arrhythmias or protein-losing enteropathy) were identified through 12 years of age. The primary study outcome was right ventricular ejection fraction (RVEF) by CMR, and primary analyses were according to shunt type received. Multivariable linear and Cox regression models were created for RVEF by CMR and post-Fontan transplant-free survival. RESULTS: Among 549 participants enrolled in SVR, 237 of 313 (76%; 60.7% male) transplant-free survivors (mBTTS, 105 of 147; RVPAS, 129 of 161; both, 3 of 5) participated in SVRIII. RVEF by CMR was similar in the shunt groups (RVPAS, 51±9.6 [n=90], and mBTTS, 52±7.4 [n=75]; P =0.43). The RVPAS and mBTTS groups did not differ in transplant-free survival by 12 years of age (163 of 277 [59%] versus 144 of 267 [54%], respectively; P =0.11), percentage predicted peak V o 2 for age and sex (74±18% [n=91] versus 72±18% [n=84]; P =0.71), or percentage predicted work rate for size and sex (65±20% versus 64±19%; P =0.65). The RVPAS versus mBTTS group had a higher cumulative incidence of protein-losing enteropathy (5% versus 2%; P =0.04) and of catheter interventions (14 versus 10 per 100 patient-years; P =0.01), but had similar rates of other complications. CONCLUSIONS: By 12 years after the Norwood operation, shunt type has minimal association with RVEF, peak V o 2 , complication rates, and transplant-free survival. RVEF is preserved among the subgroup of survivors who underwent CMR assessment. Low transplant-free survival, poor exercise performance, and accruing morbidities highlight the need for innovative strategies to improve long-term outcomes in patients with hypoplastic left heart syndrome. REGISTRATION: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT0245531.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Physiology (medical),Cardiology and Cardiovascular Medicine

Reference19 articles.

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3. Design and rationale of a randomized trial comparing the Blalock–Taussig and right ventricle–pulmonary artery shunts in the Norwood procedure

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