Results of surgery for Ebstein anomaly: a multicenter study from the European Congenital Heart Surgeons Association.
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%), 1(1/2) 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, 1(1/2) 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 1(1/2) ventricle repair or palliative procedures. Operative mortality did not differ significantly among repair, replacement, and 1(1/2) ventricle repair but was associated with palliative procedures for severe disease early in life, young age being the only independent predictor of operative death.
Sarris
George E
GE
Onassis Cardiac Surgery Center, Athens, Greece. gsarris@hol.gr
Giannopoulos
Nikos M
NM
Tsoutsinos
Alexander J
AJ
Chatzis
Andreas K
AK
Kirvassilis
George
G
Brawn
William J
WJ
Comas
Juan V
JV
Corno
Antonio F
AF
Di Carlo
Duccio
D
Fragata
Josè
J
Hraska
Victor
V
Jacobs
Jeffrey P
JP
Krupianko
Sofia
S
Sairanen
Heikki
H
Stellin
Giovanni
G
Urban
Andreas
A
Ziemer
Gerhard
G
D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D002648:Child; D002675:Child, Preschool; D004437:Ebstein Anomaly / Q000401:mortality / Q000601:surgery*; D005060:Europe; D005260:Female; D006351:Heart Valves / Q000601:surgery; D006352:Heart Ventricles / Q000601:surgery; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D010166:Palliative Care; D012189:Retrospective Studies; D016896:Treatment Outcome