Current spectrum of surgical procedures performed for Ebstein's malformation: An analysis of the society of thoracic surgeons congenital heart surgery database

Ryan R Davies, Sara K. Pasquali, Marshall L. Jacobs, Jeffrey J. Jacobs, Amelia S. Wallace, Christian Pizarro

Research output: Contribution to journalArticle

16 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1703-1710
Number of pages8
JournalAnnals of Thoracic Surgery
Volume96
Issue number5
DOIs
StatePublished - Nov 2013

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine

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