Heart failure after the Norwood procedure: An analysis of the Single Ventricle Reconstruction Trial

William T Mahle¹, Chenwei Hu², Felicia Trachtenberg², JonDavid Menteer³, Steven J Kindel⁴, Anne I Dipchand⁵, Marc E Richmond⁶, Kevin P Daly⁷, Heather T Henderson⁸, Kimberly Y Lin⁹, Michael McCulloch¹⁰, Ashwin K Lal¹¹, Kurt R Schumacher¹², Jeffrey P Jacobs¹³, Andrew M Atz¹⁴, Chet R Villa¹⁵, Kristin M Burns¹⁶, Jane W Newburger⁷; Pediatric Heart Network Investigators

Affiliations

¹ Children's Healthcare of Atlanta and Department of Pediatrics, Division of Cardiology Emory University Atlanta, GA (W.T.M). Electronic address: wmahle@emory.edu.
² New England Research Institutes, Watertown, MA (F.T., C.H.).
³ Children's Hospital Los Angeles and Department of Pediatrics, Division of Cardiology University of Southern California, Los Angeles, CA (J.M.).
⁴ Children's Hospital of Wisconsin, Milwaukee and Department of Pediatrics, Division of Cardiology University of Wisconsin Milwaukee, WI (S.J.K.).
⁵ The Hospital for Sick Children and Department of Pediatrics, Division of Cardiology University of Toronto, Toronto, Ontario (A.I.D.).
⁶ Morgan Stanley Children's Hospital of New York Presbyterian Columbia University Medical Center and Department of Pediatrics, Division of Cardiology Columbia University, New York, NY (M.E.R.).
⁷ Boston Children's Hospital and Department of Pediatrics Cardiology Harvard School of Medicine, Boston, MA (K.PD., J.W.N.).
⁸ Duke University Hospital and Department of Pediatrics, Division of Cardiology Duke University, Durham, NC (H.T.H.).
⁹ Children's Hospital of Philadelphia and Department of Pediatrics, Division of Cardiology University of Pennsylvania, Philadelphia, PA (K.L.).
¹⁰ Alfred I. DuPont Hospital for Children and Department of Pediatrics, Division of Cardiology Thomas Jefferson University, Wilmington, DE (M.M.).
¹¹ Primary Children's Medical Center and Department of Pediatrics, Division of Cardiology University of Utah, Salt Lake City, UT (A.K.L.).
¹² University of Michigan Health System and Department of Pediatrics, Division of Cardiology University of Michigan, Ann Arbor, MI (K.S.).
¹³ Johns Hopkins All Children's Heart Institute and Department of Surgery, Division of Cardiothoracic Surgery, St. Petersburg, FL (J.P.J.).
¹⁴ Department of Pediatrics, Division of Cardiology Medical University of South Carolina, Charleston, SC (A.M.A.).
¹⁵ Cincinnati Children's Hospital Medical Center and Department of Pediatrics, Division of Cardiology University of Cincinnati, Cincinnati, OH (C.R.V.).
¹⁶ National Heart, Lung, and Blood Institute, Bethesda, MD (K.M.B.).

PMID: 29571602
PMCID: PMC6015519
DOI: 10.1016/j.healun.2018.02.009

Heart failure after the Norwood procedure: An analysis of the Single Ventricle Reconstruction Trial

William T Mahle et al. J Heart Lung Transplant. 2018 Jul.

. 2018 Jul;37(7):879-885.

doi: 10.1016/j.healun.2018.02.009. Epub 2018 Feb 16.

Authors

Affiliations

¹ Children's Healthcare of Atlanta and Department of Pediatrics, Division of Cardiology Emory University Atlanta, GA (W.T.M). Electronic address: wmahle@emory.edu.
² New England Research Institutes, Watertown, MA (F.T., C.H.).
³ Children's Hospital Los Angeles and Department of Pediatrics, Division of Cardiology University of Southern California, Los Angeles, CA (J.M.).
⁴ Children's Hospital of Wisconsin, Milwaukee and Department of Pediatrics, Division of Cardiology University of Wisconsin Milwaukee, WI (S.J.K.).
⁵ The Hospital for Sick Children and Department of Pediatrics, Division of Cardiology University of Toronto, Toronto, Ontario (A.I.D.).
⁶ Morgan Stanley Children's Hospital of New York Presbyterian Columbia University Medical Center and Department of Pediatrics, Division of Cardiology Columbia University, New York, NY (M.E.R.).
⁷ Boston Children's Hospital and Department of Pediatrics Cardiology Harvard School of Medicine, Boston, MA (K.PD., J.W.N.).
⁸ Duke University Hospital and Department of Pediatrics, Division of Cardiology Duke University, Durham, NC (H.T.H.).
⁹ Children's Hospital of Philadelphia and Department of Pediatrics, Division of Cardiology University of Pennsylvania, Philadelphia, PA (K.L.).
¹⁰ Alfred I. DuPont Hospital for Children and Department of Pediatrics, Division of Cardiology Thomas Jefferson University, Wilmington, DE (M.M.).
¹¹ Primary Children's Medical Center and Department of Pediatrics, Division of Cardiology University of Utah, Salt Lake City, UT (A.K.L.).
¹² University of Michigan Health System and Department of Pediatrics, Division of Cardiology University of Michigan, Ann Arbor, MI (K.S.).
¹³ Johns Hopkins All Children's Heart Institute and Department of Surgery, Division of Cardiothoracic Surgery, St. Petersburg, FL (J.P.J.).
¹⁴ Department of Pediatrics, Division of Cardiology Medical University of South Carolina, Charleston, SC (A.M.A.).
¹⁵ Cincinnati Children's Hospital Medical Center and Department of Pediatrics, Division of Cardiology University of Cincinnati, Cincinnati, OH (C.R.V.).
¹⁶ National Heart, Lung, and Blood Institute, Bethesda, MD (K.M.B.).

PMID: 29571602
PMCID: PMC6015519
DOI: 10.1016/j.healun.2018.02.009

Abstract

Background: Heart failure results in significant morbidity and mortality in young children with hypoplastic left heart syndrome (HLHS) after the Norwood procedure.

Methods: We studied subjects enrolled in the prospective Single Ventricle Reconstruction (SVR) Trial who survived to hospital discharge after a Norwood operation and were followed up to age 6 years. The primary outcome was heart failure, defined as heart transplant listing after Norwood hospitalization, death attributable to heart failure, or symptomatic heart failure (New York Heart Association [NYHA] Class IV). Multivariate modeling was undertaken using Cox regression methodology to determine variables associated with heart failure.

Results: Of the 461 subjects discharged home following a Norwood procedure, 66 (14.3%) met the criteria for heart failure. Among these, 15 died from heart failure, 39 were listed for transplant (22 had a transplant, 12 died after listing, and 5 were alive and not yet transplanted), and 12 had NYHA Class IV heart failure but were never listed. The median age at heart failure identification was 1.28 (interquartile range 0.30 to 4.69) years. Factors associated with early heart failure included post-Norwood lower fractional area change, need for extracorporeal membrane oxygenation, non-Hispanic ethnicity, Norwood perfusion type, and total support time (p < 0.05).

Conclusions: By 6 years of age, heart failure developed in nearly 15% of children after the Norwood procedure. Although transplant listing was common, many patients died from heart failure before receiving a transplant or without being listed. Shunt type did not impact the risk of developing heart failure.

Trial registration: ClinicalTrials.gov NCT00115934.

Keywords: Norwood procedure; cardiac surgery; congenital heart defect; congenital heart disease; single ventricle.

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Conflict of interest statement

Disclosure Statement: The authors have no conflicts of interest to disclose.

Figures

**Figure 1**
Diagram depicting clinical pathways for subjects with advanced heart failure.

**Figure 2**
Freedom from heart failure using all available follow-up data until 6 years (72 Months). Heart failure was defined as listing for heart transplantation, heart failure death, or NYHA Heart Failure Class IV.

See this image and copyright information in PMC

References

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Heart failure after the Norwood procedure: An analysis of the Single Ventricle Reconstruction Trial

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Heart failure after the Norwood procedure: An analysis of the Single Ventricle Reconstruction Trial

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