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Related Experiment Videos

Outcome in neonates with Ebstein's anomaly.

D S Celermajer1, S Cullen, I D Sullivan

  • 1Cardiothoracic Unit, Hospital for Sick Children, London, England.

Journal of the American College of Cardiology
|April 1, 1992
PubMed
Summary

Neonatal Ebstein's anomaly has high early mortality. A new echocardiographic grading system effectively predicts patient survival and risk of late complications, aiding prognostic stratification.

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Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Echocardiography

Background:

  • Ebstein's anomaly is a rare congenital heart defect affecting the tricuspid valve.
  • Neonatal presentation often involves cyanosis and significant morbidity.
  • Outcomes for affected neonates historically show high mortality rates.

Purpose of the Study:

  • To review the presentation and outcomes of neonates with Ebstein's anomaly.
  • To develop and validate a new echocardiographic grading system for severity.
  • To identify predictors of mortality and stratify prognosis.

Main Methods:

  • Retrospective review of 50 patients diagnosed with neonatal Ebstein's anomaly (1961-1990).
  • Development of a novel echocardiographic grading scale (1-4) based on chamber/chamber ratios.

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  • Multivariate analysis of clinical and echocardiographic features to predict outcomes.

    • Main Results:

    • High early mortality (18% neonatal deaths) and significant late deaths (61% 10-year survival).
    • Cyanosis was the most common presentation (80%); 54% had associated cardiac defects.
    • The new echocardiographic grade strongly correlated with mortality: Grade 4 had 100% cardiac death.

    Conclusions:

    • Neonates with Ebstein's anomaly face substantial early and late mortality risks.
    • The devised echocardiographic grading system is a powerful independent predictor of death.
    • This grading allows for effective prognostic stratification in neonatal Ebstein's anomaly.