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.
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 neonatalpatients (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.
Authors: Steven P Goldberg; Ryan C Jones; Umar S Boston; Lauren M Haddad; Glenn T Wetzel; Thomas K Chin; Christopher J Knott-Craig Journal: World J Pediatr Congenit Heart Surg Date: 2011-10-01
Authors: Meindert Palmen; Peter L de Jong; Loes M A Klieverik; Angelique C Venema; Folkert J Meijboom; Ad J J C Bogers Journal: Eur J Cardiothorac Surg Date: 2008-05-01 Impact factor: 4.191
Authors: Takeshi Shinkawa; Anastasios C Polimenakos; Carlen A Gomez-Fifer; John R Charpie; Jennifer C Hirsch; Eric J Devaney; Edward L Bove; Richard G Ohye Journal: J Thorac Cardiovasc Surg Date: 2009-09-22 Impact factor: 5.209
Authors: Morgan L Brown; Joseph A Dearani; Gordon K Danielson; Frank Cetta; Heidi M Connolly; Carole A Warnes; Zhuo Li; David O Hodge; David J Driscoll Journal: J Am Coll Cardiol Date: 2008-08-05 Impact factor: 24.094
Authors: David A Ashburn; Eugene H Blackstone; Winfield J Wells; Richard A Jonas; Frank A Pigula; Peter B Manning; Gary K Lofland; William G Williams; Brian W McCrindle Journal: J Thorac Cardiovasc Surg Date: 2004-04 Impact factor: 5.209
Authors: A Carpentier; S Chauvaud; L Macé; J Relland; S Mihaileanu; J P Marino; B Abry; P Guibourt Journal: J Thorac Cardiovasc Surg Date: 1988-07 Impact factor: 5.209
Authors: Tristan K W Ramcharan; Donna A Goff; Christopher E Greenleaf; Suhair O Shebani; Jorge D Salazar; Antonio F Corno Journal: Pediatr Cardiol Date: 2022-04-23 Impact factor: 1.838