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

George E. Sarris, Nikos M. Giannopoulos, Alexander J. Tsoutsinos, Andreas K. Chatzis, George Kirvassilis, William J. Brawn, Juan V. Comas, Antonio F. Corno, Duccio Di Carlo, Josè Fragata, Victor Hraska, Jeffrey P. Jacobs, Sofia Krupianko, Heikki Sairanen, Giovanni Stellin, Andreas Urban, Gerhard Ziemer

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Abstract

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.

Original languageEnglish
JournalJournal of Thoracic and Cardiovascular Surgery
Volume132
Issue number1
DOIs
Publication statusPublished - Jul 2006

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Ebstein Anomaly
Multicenter Studies
Mortality

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery

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Sarris, G. E., Giannopoulos, N. M., Tsoutsinos, A. J., Chatzis, A. K., Kirvassilis, G., Brawn, W. J., ... Ziemer, G. (2006). Results of surgery for Ebstein anomaly: A multicenter study from the European Congenital Heart Surgeons Association. Journal of Thoracic and Cardiovascular Surgery, 132(1). https://doi.org/10.1016/j.jtcvs.2005.10.062

Results of surgery for Ebstein anomaly : A multicenter study from the European Congenital Heart Surgeons Association. / Sarris, George E.; Giannopoulos, Nikos M.; Tsoutsinos, Alexander J.; Chatzis, Andreas K.; Kirvassilis, George; Brawn, William J.; Comas, Juan V.; Corno, Antonio F.; Di Carlo, Duccio; Fragata, Josè; Hraska, Victor; Jacobs, Jeffrey P.; Krupianko, Sofia; Sairanen, Heikki; Stellin, Giovanni; Urban, Andreas; Ziemer, Gerhard.

In: Journal of Thoracic and Cardiovascular Surgery, Vol. 132, No. 1, 07.2006.

Research output: Contribution to journalArticle

Sarris, GE, Giannopoulos, NM, Tsoutsinos, AJ, Chatzis, AK, Kirvassilis, G, Brawn, WJ, Comas, JV, Corno, AF, Di Carlo, D, Fragata, J, Hraska, V, Jacobs, JP, Krupianko, S, Sairanen, H, Stellin, G, Urban, A & Ziemer, G 2006, 'Results of surgery for Ebstein anomaly: A multicenter study from the European Congenital Heart Surgeons Association', Journal of Thoracic and Cardiovascular Surgery, vol. 132, no. 1. https://doi.org/10.1016/j.jtcvs.2005.10.062
Sarris, George E. ; Giannopoulos, Nikos M. ; Tsoutsinos, Alexander J. ; Chatzis, Andreas K. ; Kirvassilis, George ; Brawn, William J. ; Comas, Juan V. ; Corno, Antonio F. ; Di Carlo, Duccio ; Fragata, Josè ; Hraska, Victor ; Jacobs, Jeffrey P. ; Krupianko, Sofia ; Sairanen, Heikki ; Stellin, Giovanni ; Urban, Andreas ; Ziemer, Gerhard. / Results of surgery for Ebstein anomaly : A multicenter study from the European Congenital Heart Surgeons Association. In: Journal of Thoracic and Cardiovascular Surgery. 2006 ; Vol. 132, No. 1.
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abstract = "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.",
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AU - Sarris, George E.

AU - Giannopoulos, Nikos M.

AU - Tsoutsinos, Alexander J.

AU - Chatzis, Andreas K.

AU - Kirvassilis, George

AU - Brawn, William J.

AU - Comas, Juan V.

AU - Corno, Antonio F.

AU - Di Carlo, Duccio

AU - Fragata, Josè

AU - Hraska, Victor

AU - Jacobs, Jeffrey P.

AU - Krupianko, Sofia

AU - Sairanen, Heikki

AU - Stellin, Giovanni

AU - Urban, Andreas

AU - Ziemer, Gerhard

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N2 - 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.

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