Ebstein's anomaly: late results of surgical correction

Between 1969 and 1976, 10 severely disabled patients with Ebstein's anomaly were operated on at the National Heart Hospital using replacement of the tricuspid valve with mounted aortic homografts [9] or dura mater valve [1], plication of the atrialized right ventricle [10], and closure of an associated interatrail communication. 7 survivors have been followed up from 2 to 9 yr. 5 are asymptomatic. Symptomatic tricuspid regurgitation developed in 2 patients; from a paravalvar leak of the tricuspid homograft in one, and in the other calcification with stenosis in an irradiated homograft, 2.5 and 7.5 yr later. Permanent pacemakers were needed in 2 patients, 3 wk and 18 mth after surgery for symptomatic heart block. Despite corrective surgery for the structural abnormalities in Ebstein, late results remain influenced by disorders of rhythm and conduction disturbances, degenerative changes in the valves used for replacement and the intrinsic primary myocardial disease. Such "corrective" procedures in patients with Ebstein's anomaly can result in maintained symptomatic improvement but must be regarded as palliative surgery.