Current spectrum of surgical procedures performed for Ebstein's malformation: an analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database.
BACKGROUND
Ebstein's malformation is a rare congenital cardiac anomaly. Available data are limited to individual reports demonstrating highly variable approaches. We sought to understand the spectrum of surgical treatment of Ebstein's anomaly across institutions.


METHODS
A retrospective review of surgical procedures performed on patients with primary diagnosis of Ebstein's malformation (2002 through 2009) in The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) was conducted.


RESULTS
A total of 595 operations on 498 patients with Ebstein's anomaly were included: 116 on neonates (19%), 122 on infants (21%), 264 on children (44%), and 93 on adults (16%). Average annual institutional case volumes were low (median, 1 per year; range, 0 to 8 per year). Neonates had a high rate of palliative procedures: systemic-to-pulmonary artery shunts with or without tricuspid valve closure (43; 37.1%) and tricuspid valve closure (10; 8.6%); Ebstein's repair or tricuspid valvuloplasty was performed in 32%. The most common procedures among infants were superior cavopulmonary connections (62; 50.8%) and systemic-to-pulmonary shunt (10; 8.2%). Among older patients, procedures were primarily in three categories: tricuspid valve surgery (children, 54.5%; adults, 68.8%), arrhythmia procedures (children, 8.7%; adults, 17.3%), and Fontan (children, 14.8%). In-hospital mortality was high in neonatal patients (23.4%) in comparison with infants (4.1%), children (0.7%), and adults (1.1%).


CONCLUSIONS
Surgery for Ebstein's anomaly consists of a wide range of procedures, with low individual institutional volumes. Mortality is highest among neonates. A prospective multicenter inception cohort study would be valuable to better define indications for specific strategies of surgical management.
Nemours Cardiac Center, Nemours/A.I. duPont Hospital for Children, Wilmington, Delaware. Electronic address: rdavies@nemours.org.
Ryan R
RR
Davies
Sara K
SK
Pasquali
Marshall L
ML
Jacobs
Jeffrey J
JJ
Jacobs
Amelia S
AS
Wallace
Christian
C
Pizarro
D000328:Adult; D006348:Cardiac Surgical Procedures / Q000379:methods; D002648:Child; D016208:Databases, Factual; D004437:Ebstein Anomaly / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D012189:Retrospective Studies; D012955:Societies, Medical; D013903:Thoracic Surgery
21