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George E. Sarris
William J. Brawn
Juan V. Comas
Antonio F. Corno
Duccio Di Carlo
Josè Fragata
Jeffrey P. Jacobs
Heikki Sairanen
Giovanni Stellin
Andreas Urban
Gerhard Ziemer
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Right arrow Congenital - cyanotic
Right arrow Valve disease

J Thorac Cardiovasc Surg 2006;132:50-57
© 2006 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Results of surgery for Ebstein anomaly: A multicenter study from the European Congenital Heart Surgeons Association

George E. Sarris, MD a , * , Nikos M. Giannopoulos, MD a , Alexander J. Tsoutsinos, MD a , Andreas K. Chatzis, MD a , George Kirvassilis, MD a , William J. Brawn, MD b , Juan V. Comas, MD c , Antonio F. Corno, MD d , Duccio Di Carlo, MD e , Josè Fragata, MD f , Victor Hraska, MD g , Jeffrey P. Jacobs, MD h , Sofia Krupianko, MD i , Heikki Sairanen, MD j , Giovanni Stellin, MD k , Andreas Urban, MD l , Gerhard Ziemer, MD m European Congenital Heart Surgeons Association

a Onassis Cardiac Surgery Center, Athens, Greece
b Birmingham Children's Hospital, Birmingham, United Kingdom
c Pediatric Heart Institute, Hospital Universitario "12 de Octubre," Madrid, Spain
d data from CHUV, Lausanne, Switzerland, author at University of Liverpool, Alder Hey Royal Children's Hospital, United Kingdom
e Ospedale Pediatrico Bambino Gesu, Rome, Italy
f Hospital de Santa Marta, Lisbon, Portugal
g Eppendorf University Hospital, Hamburg, Germany
h The Congenital Heart Institute of Florida, University of South Florida, All Children's Hospital/Children's Hospital of Tampa, St Petersburg and Tampa, Fla
i Bakulev Center for Cardiovascular Surgery, Moscow, Russia
j Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland
k University of Padova, Padova, Italy
l Deutsches Kinderherzzentrum, St Augustin, Germany
m Eberhard Karls University, Tubingen, Germany.

Read at the Eighty-fifth Annual Meeting of The American Association for Thoracic Surgery, San Francisco, Calif, April 10-13, 2005.

Received for publication April 14, 2005; revisions received September 4, 2005; accepted for publication October 10, 2005.

* Address for reprints: George E. Sarris, MD, Onassis Cardiac Surgery Center, 356 Sygrou Ave, Athens, Greece 176 74. (Email: gsarris{at}hol.gr).

OBJECTIVE: Since most centers' experience with Ebstein anomaly is limited, we sought to analyze the collective experience of participating institutions of the European Congenital Heart Surgeons Association with surgery for this rare malformation.

METHODS: The records of all 150 patients (median age 6.4 years) who underwent surgery for Ebstein anomaly in the 13 participating Association centers between January 1992 and January 2005 were reviewed retrospectively. Patients with congenitally corrected transposition were excluded.

RESULTS: Most patients (81%) had Ebstein disease type B or C and significant functional impairment (61% in New York Heart Association class III or IV) and 16% had prior operations. Surgical procedures (n = 179) included valve replacement (n = 60, 33.5%), valve repair (n = 49, 27.3%), 11/2 ventricle repair (n = 46, 25.6%), palliative shunt (n = 13, 7.26%), and other complex procedures (n = 11, 6.14%). There were 20 hospital deaths (operative mortality 13.3%) after valve replacement in 5 patients, valve repair in 3, 11/2 ventricle repair in 7, palliative procedures in 3, and miscellaneous procedures in 2. Younger age and palliative procedures were univariate risk factors for operative death, but only age was an independent predictor on multivariable analysis.

CONCLUSIONS: Most patients coming to surgery presented in childhood and were significantly symptomatic. More than half underwent valve replacement or repair, but a considerable proportion had severe disease necessitating 11/2 ventricle repair or palliative procedures. Operative mortality did not differ significantly among repair, replacement, and 11/2 ventricle repair but was associated with palliative procedures for severe disease early in life, young age being the only independent predictor of operative death.

Abbreviations and Acronyms ASD = atrial septal defect; ECHSA = European Congenital Heart Surgeons Association; RV = right ventricle/ventricular; TV = tricuspid valve




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