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Hypoplastic Left Heart Syndrome: Exploring a Paradigm Shift in Favor of Surgery

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Abstract

We hypothesized that enthusiasm for surgery increased for infants with hypoplastic left heart syndrome (HLHS) at Columbia University Medical Center (CUMC) between 1995 and 2012. We sought to identify factors that engendered this paradigm shift. Confidential surveys were distributed to providers at CUMC in 1995 and 2012 to measure enthusiasm for surgical intervention for HLHS. Surgical preference scores are presented as median [interquartile range]. Surveys were completed by 99/176 providers (56 % response rate) in 1995 and 153/267 (57 %) in 2012. The median surgical preference score for infants with HLHS increased from 35 [25–45] in 1995 to 45 [35–50] in 2012, P < 0.001. 53 %, 95 % CI [42, 64] of respondents recommended surgical intervention for a ward of the court in 1995 compared to 81 % [73, 89] in 2012, P < 0.001. In 2012, 64 % [53, 75] of respondents were more likely to recommend surgery than 10 years prior. The percentage of respondents who saw good outcomes following three-stage repair increased from 49 % [38, 60] in 1995 to 84 % [78, 90] in 2012, P < 0.001. The majority believed that parents should have the option of comfort care, 91 % [85, 97] in 1995 and 85 % [79, 91] in 2012, P = 0.06. In both eras, prematurity and additional surgical problems dissuaded providers from recommending surgical intervention. Despite the fact that most providers have seen good outcomes and now recommend surgery for infants with HLHS, the majority of providers still believe that the option of comfort care should be available to families.

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Acknowledgments

This study was supported in part by an internal grant from the pediatric residency program in the Department of Pediatrics at New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center.

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Authors and Affiliations

  1. Division of Pediatric Cardiology, Department of Pediatrics, New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center, 3959 Broadway, CHN-253, New York, NY, 10032, USA

    Erin A. Paul MD & Thomas J. Starc MD, MPH

  2. Division of Neonatology and Bioethics, Department of Pediatrics, New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center, 3959 Broadway, CHN-253, New York, NY, 10032, USA

    Kristina Orfali PhD

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  1. Erin A. Paul MD
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  2. Kristina Orfali PhD
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  3. Thomas J. Starc MD, MPH
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Correspondence to Erin A. Paul MD.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. For this type of study, formal consent is not required. This article does not contain any studies with animals performed by any of the authors.

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Paul, E.A., Orfali, K. & Starc, T.J. Hypoplastic Left Heart Syndrome: Exploring a Paradigm Shift in Favor of Surgery. Pediatr Cardiol 37, 1446–1452 (2016). https://doi.org/10.1007/s00246-016-1455-y

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