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Comparative Outcomes and Risk Analysis after Cone Repair or Tricuspid Valve Replacement for Ebstein’s Anomaly

  • Author Footnotes
    ∗ co-first authors
    Rebekah Boyd
    Footnotes
    ∗ co-first authors
    Affiliations
    Department of Surgery, Division of Cardiac, Thoracic and Vascular Surgery, Section of Pediatric and Congenital and Cardiac Surgery, Morgan Stanley Children Hospital -New York Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
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  • Author Footnotes
    ∗ co-first authors
    David Kalfa
    Footnotes
    ∗ co-first authors
    Affiliations
    Department of Surgery, Division of Cardiac, Thoracic and Vascular Surgery, Section of Pediatric and Congenital and Cardiac Surgery, Morgan Stanley Children Hospital -New York Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
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  • Stephanie Nguyen
    Affiliations
    Department of Surgery, Division of Cardiac, Thoracic and Vascular Surgery, Section of Pediatric and Congenital and Cardiac Surgery, Morgan Stanley Children Hospital -New York Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
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  • Matan Setton
    Affiliations
    Division of Pediatric Cardiology, Morgan Stanley Children Hospital -New York Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
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  • Amee Shah
    Affiliations
    Division of Pediatric Cardiology, Morgan Stanley Children Hospital -New York Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
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  • John Karamichalis
    Affiliations
    Department of Surgery, Division of Cardiac, Thoracic and Vascular Surgery, Section of Pediatric and Congenital and Cardiac Surgery, Morgan Stanley Children Hospital -New York Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
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  • Matthew Lewis
    Affiliations
    Division of Cardiology, New York Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
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  • Noa Zemer Wassercug
    Affiliations
    Division of Cardiology, New York Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
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  • Marlon Rosenbaum
    Affiliations
    Division of Cardiology, New York Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
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  • Emile Bacha
    Correspondence
    Corresponding Author: Emile Bacha, MD, Columbia University College of Physicians and Surgeons, New York-Presbyterian Morgan Stanley Children's Hospital, 3959 Broadway, CHN-274, New York, NY 10032, Phone: 212 (305)-2688; Fax: 212 (305)-4408,
    Affiliations
    Department of Surgery, Division of Cardiac, Thoracic and Vascular Surgery, Section of Pediatric and Congenital and Cardiac Surgery, Morgan Stanley Children Hospital -New York Presbyterian Hospital, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
    Search for articles by this author
  • Author Footnotes
    ∗ co-first authors
Open AccessPublished:March 17, 2023DOI:https://doi.org/10.1016/j.xjon.2023.03.004
Comparative Outcomes and Risk Analysis after Cone Repair or Tricuspid Valve Replacement for Ebstein’s Anomaly
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      Abstract:

      Objective

      Ebstein’s anomaly (EA) is a rare congenital heart malformation whose surgical and medical management is still controversial. The cone repair has transformed surgical outcomes in many of these patients. We aimed to present our results on outcomes of EA patients that underwent a cone repair or tricuspid valve replacement (TVR).

      Methods

      85 patients who underwent a cone repair (mean age 16.5 year) or TVR (mean age 40.8 years) between 2006-2021 were included. Univariate, multivariate, and Kaplan-Meier analyses evaluated operative and long-term outcomes.

      Results

      Residual/recurrent greater than mild-to-moderate TR at discharge was higher after cone repair compared to TVR (36% vs 5%; p=0.010). However, at last follow-up, the risk of greater than mild-to-moderate TR was not different between groups (35% in cone vs 37% in TVR; p=0.786). TVR patients had a higher risk of TV reoperation (37% vs 9%; p=0.005) and tricuspid stenosis (21% vs 0%; p = 0.002) compared to cone patients. Kaplan-Meier freedom from reintervention was 97%, 91% and 91% at 2, 4, and 6 years after cone repair, and 84%, 74%, and 68% at 2, 4, and 6 years after TVR (p = 0.0191). At last follow-up, RV function was significantly worse from baseline in the TVR group (p = 0.0294). There were no statistical differences between age-stratified cohorts or surgeon volume in the cone repair group.

      Conclusions

      The cone procedure offers excellent results, with stable TV function and low reintervention and death rates at last follow-up. The rate of greater than mild-to-moderate residual TR at discharge was higher after cone compared to TVR but this did not expose to a higher risk of reoperation or death at last follow-up. TVR was associated with a significantly higher risk of TV reoperation, TV stenosis and worse RV function at last follow-up.

      Graphical abstract

      Figure thumbnail fx1
      Graphical Abstract

      Keywords

      Glossary of Abbreviations:

      EA (Ebstein’s anomaly), RV (Right ventricle), TR (Tricuspid regurgitation), TV (Tricuspid valve), TVR (Tricuspid valve replacement), TS (Tricuspid stenosis)

      Article info

      Publication history

      Accepted: March 7, 2023
      Received: July 18, 2022

      Publication stage

      In Press Journal Pre-Proof

      Footnotes

      Disclosure Statement:

      The authors have nothing to disclose

      Funding Statement:

      No external funding was received for this work.

      Informed Consent Statement:

      Informed written consent was waived due to the retrospective nature of this study.

      Central Picture Legend: Cone repair shows significantly greater freedom from reintervention compared to TVR.

      Central Message:

      Cone repair is associated with stable TV function long term and a significantly lower risk of TV stenosis, TV reoperation, and RV function worsening compared to valve replacement.

      Perspective Statement:

      Cone repair has become the gold standard for TV repair for Ebstein’s anomaly. In addition to stable long term TV function and lower rates of reintervention, it also demonstrates improved right ventricular function over time compared to valve replacement. The use of cone repair might be safely extended to use in older, sicker patients to improve right ventricular remodeling.

      Identification

      DOI: https://doi.org/10.1016/j.xjon.2023.03.004

      Copyright

      © 2023 The Authors. Published by Elsevier Inc. on behalf of The American Association for Thoracic Surgery

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