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Ebstein's anomaly: a long-term study of survival
1Department of Pediatrics, University of Minnesota, Minneapolis 55455.
American Heart Journal
|May 1, 1993
Summary
Survival for Ebstein's malformation patients averages 15 years, with factors like functional class and surgery impacting outcomes. Further multi-institutional data clarifies the disease course and surgical treatment decisions.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Ebstein's malformation is a rare congenital heart defect.
- Understanding survival rates and prognostic factors is crucial for patient management.
Purpose of the Study:
- To analyze survival data for patients with Ebstein's malformation.
- To identify factors influencing survival in this patient population.
Main Methods:
- Retrospective analysis of patient data from a single institution (University of Minnesota).
- Inclusion of data from a multi-institutional study over the past decade.
- Examination of factors including NYHA functional classification, hemoglobin levels, and cardiac surgery.
Main Results:
- In a cohort of 61 patients, average survival was 15 years, with 31 patients alive.
- In a larger multi-institutional study (18,281 patients), 71 had Ebstein's malformation.
- Infant mortality was higher, with 7 of 16 infants dying in the first year; 4 of 55 older patients died, all post-cardiac surgery.
Conclusions:
- Several factors, including NYHA class, hemoglobin, atrial pressure, neonatal symptoms, and cardiac surgery, negatively impact survival.
- Data from multi-institutional studies enhance understanding of Ebstein's malformation prognosis.
- Findings aid in clinical decision-making regarding surgical interventions for Ebstein's malformation.