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Objectives <p>To investigate the long-term outcomes and risk factors for morbidity and mortality in patients with Ebstein’s anomaly, including the effects of type and timing of valve surgery.</p> Methods <p>For this retrospective, record-based study, all patients with Ebstein’s anomaly enrolled in the <i>German National Register for Congenital Heart Defects</i> up to June 2021 were eligible for inclusion.</p> Results <p>Non-surgical patients (<i>n</i> = 194/49% of 398 patients) had less tricuspid valve regurgitation (<i>p</i> &lt; 0.001) and heart failure symptoms (<i>p</i> &lt; 0.001) than surgical patients (<i>n</i> = 204/51%). Postoperative survival at 10, 20, and 30&#xa0;years was 97%, 93%, and 80%. Eighty-one (40%) patients underwent multiple surgeries. Re-operation rates were lowest in patients with first valve surgery during adolescence (<i>p</i> = 0.0076). Postoperative NYHA class &gt; I was more frequent with surgery delayed to older age (<i>p</i> &lt; 0.001). Initial corrective surgery was complicated by complete atrioventricular block (CAVB) in 17 (9%) of patients. CAVB was more likely with older age (<i>p</i> = 0.001), and tricuspid valve replacement compared to reconstruction (<i>p</i> = 0.029). CAVB was associated with all-cause death (<i>p</i> &lt; 0.001). Cone reconstruction reduced the risk of CAVB (<i>p</i> = 0.008) and tricuspid valve regurgitation (<i>p</i> &lt; 0.001) compared to monocusp reconstruction.</p> Conclusions <p>This registry-based study of Ebstein’s anomaly corroborates good surgical long-term results, while re-operation rates remain high. Patients operated before adolescence were at the highest risk of re-operation, while older age at the time of the first surgery increased the risk of CAVB. The cone reconstruction was associated with improved tricuspid valve function and a lower risk of CAVB compared to monocusp reconstructions. Choosing an optimal time window for surgery and use of the cone reconstruction may therefore further improve outcomes.</p> Graphical Abstract <p></p>

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Risk factors for morbidity and mortality in Ebstein’s anomaly: a registry-based study of 398 patients

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年3月4日

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  • Stephan Neumann,
  • Luisa K. Heneka,
  • Michael Jerosch-Herold,
  • Gerhard Schön,
  • Ulrike M. M. Bauer,
  • Michael Hübler,
  • Daniel Biermann,
  • Götz Müller,
  • Ines Kowalewski,
  • Martin Munz,
  • Rainer Kozlik-Feldmann,
  • Christoph Sinning,
  • Elvin Zengin,
  • Carsten Rickers

摘要

Objectives

To investigate the long-term outcomes and risk factors for morbidity and mortality in patients with Ebstein’s anomaly, including the effects of type and timing of valve surgery.

Methods

For this retrospective, record-based study, all patients with Ebstein’s anomaly enrolled in the German National Register for Congenital Heart Defects up to June 2021 were eligible for inclusion.

Results

Non-surgical patients (n = 194/49% of 398 patients) had less tricuspid valve regurgitation (p < 0.001) and heart failure symptoms (p < 0.001) than surgical patients (n = 204/51%). Postoperative survival at 10, 20, and 30 years was 97%, 93%, and 80%. Eighty-one (40%) patients underwent multiple surgeries. Re-operation rates were lowest in patients with first valve surgery during adolescence (p = 0.0076). Postoperative NYHA class > I was more frequent with surgery delayed to older age (p < 0.001). Initial corrective surgery was complicated by complete atrioventricular block (CAVB) in 17 (9%) of patients. CAVB was more likely with older age (p = 0.001), and tricuspid valve replacement compared to reconstruction (p = 0.029). CAVB was associated with all-cause death (p < 0.001). Cone reconstruction reduced the risk of CAVB (p = 0.008) and tricuspid valve regurgitation (p < 0.001) compared to monocusp reconstruction.

Conclusions

This registry-based study of Ebstein’s anomaly corroborates good surgical long-term results, while re-operation rates remain high. Patients operated before adolescence were at the highest risk of re-operation, while older age at the time of the first surgery increased the risk of CAVB. The cone reconstruction was associated with improved tricuspid valve function and a lower risk of CAVB compared to monocusp reconstructions. Choosing an optimal time window for surgery and use of the cone reconstruction may therefore further improve outcomes.

Graphical Abstract