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26. RESULTS OF SURGERY FOR EBSTEIN’S ANOMALY: A MULTI-CENTER STUDY FROM THE EUROPEAN CONGENITAL HEART SURGEONS ASSOCIATION.
Nickolas Miltiadis Giannopoulos, Andrew Konstantine Chatzis, Alexandros Yannis Tsoutsinos, George Vassilis Kirvassilis, George Elias Sarris; Athens, Greece
Objective: As Ebstein’s anomaly is a rare congenital malformation, most centres’ experience with this lesion is limited to a small number of patients accumulated over decades. Therefore, we sought to analyze the collective experience of participating institutions of the European Congenital Heart Surgeons Association (ECHSA) with surgery for Ebstein’s anomaly in the current era.
Methods: The records of all 152 patients ( 51% female), median age 6 years, with diagnosis of Ebstein’s anomaly who underwent surgical therapy in one of the 11 participating ECHSA centres between 1/1992 and 7/2004 were reviewed. Patients with congenitally corrected transposition and Ebstenoid malformation of the systemic atrioventricular valve were excluded. Most patients (90%) had Ebstein disease type B or C associated with moderate to severe tricuspid valve displacement, mild to moderate cyanosis (median O2Sat 88%), increased cardiothoracic ratio ( median 66%), moderately (50%) or severely (70%) impaired right ventricular function, and significant functional impairment (52% in NYHA class III or IV). First time surgical procedures performed in 126 patients included tricuspid valve (TV) replacement in 42, repair in 32, 1½ ventricle repair in 30, palliative procedures (e.g. arteriopulmonary or Glenn shunts, hemi-Fontan) were performed in 12 cases and miscellaneous procedures in 10 patients. In 28 patients the procedure performed was a reoperation, including TV replacement in 10, TV repair in 3, 1½ ventricle operation in 7, and palliation in 5 and other procedures in 3.
Results: There were 20 hospital deaths (operative mortality 13%). 12(60%) deaths cardiac 8(40%) non-cardiac, 4(20%) in valve replacement cases, 3(15%) valve repair, 2(10%) 11/2 ventricle repair, 5(25%) palliative procedures, 6(30%) in miscellaneous procedures. Median mechanical ventillation time was 1 day, median ICU stay 2 days and median hospital stays 15 days.
Conclusions: Most patients coming to surgery in participating European centres present in childhood, are at least mildly cyanotic and significantly symptomatic, have anatomically moderate to severe disease, with 18% has had prior cardiac surgery. More than half of the patients undergo valve replacement (35%) or repair (18%), but still a considerable proportion (43%) have severe disease necessitating 1&½ ventricle repair or other palliative procedures. Operative mortality is not low (13%), and is mostly associated with palliative procedures for severe Ebstein’s disease.
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