• Ryan R Davies, Sara K Pasquali, Marshall L Jacobs, Jeffrey J Jacobs, Amelia S Wallace, and Christian Pizarro.
• Nemours Cardiac Center, Nemours/A.I. duPont Hospital for Children, Wilmington, Delaware. Electronic address: rdavies@nemours.org.
• Ann. Thorac. Surg. 2013 Nov 1; 96 (5): 1703-9; discussion 1709-10.

BackgroundEbstein'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.MethodsA 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.ResultsA 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%).ConclusionsSurgery 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.Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

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