TRICUSPID-VALVE ABNORMALITIES IN PATIENTS WITH ATRIOVENTRICULAR DISCORDANCE - SURGICAL IMPLICATIONS

From 1975 to 1990, 90 patients (age, 6 months to 30 years; mean, 9.1 y ears) underwent operation for defects associated with atrioventricular discordance. Twenty-one had an anatomically abnormal tricuspid (syste mic) atrioventricular valve (SAVV) of the following types: Ebstein, 11 ; straddling, 6; and dysplastic, 4. Sixteen valves were regurgitant: r egurgitation was trivial to mild in nine and moderate to severe in sev en. Two patients underwent a successful Fontan-type operation. None of the 5 patients with a competent SAW underwent valve repair or replace ment; 1 of these patients died. A ventricular septal defect was closed in 14 and an extracardiac valved conduit was placed in 7. Sixteen had a regurgitant valve: it was replaced in 10 and repaired in 2 (early m ortality, 25%). All 4 patients who did not undergo repair or replaceme nt of their regurgitant SAVV died. Two patients died late after repeat replacement. Four other reoperations (closure of a residual ventricul ar septal defect, SAW replacement, left ventricle-to-pulmonary artery conduit replacement, and a redo Fontan procedure) were successful. Two patients are lost to follow-up, and 9 have been followed up for from 27 to 156 months (median, 117 months). All were well when last seen. W e suggest that an abnormal regurgitant SAVV should be replaced. Altern atively, a ''double-switch'' procedure that leaves the tricuspid valve in the pulmonary circulation may be used.