Ebstein's anomaly: A long-term study of survival

Abstract

This study examines data regarding the survival of patients with Ebstein's malformation. Of 61 patients studied at the University of Minnesota, 31 are currently alive, with the average survival being 15 years. Several factors adversely affected survival: NYHA functional classification, hemoglobin level, right atrial pressure, symptoms during the neonatal period, and cardiac surgery. Data from a multiinstitutional study collected during the past decade adds information regarding survival. Of 18,281 patients in the study, 71 (0.4%) had Ebstein's malformation. Sixteen of these were among 7235 infants, and of these seven died during the first year of life. In contrast, among 55 patients over the age of 1 year, four died, each after cardiac surgery. The data help our understanding of the course of patients with Ebstein's malformation and decisions regarding surgical treatment.