CONGENITALLY CORRECTED TRANSPOSITION OF THE GREAT-ARTERIES IN THE ADULT - FUNCTIONAL STATUS AND COMPLICATIONS

Objectives. We sought to assess the clinical outcome, functional statu s and complications of adult patients with congenitally corrected tran sposition of the great arteries. Background. Congenitally corrected tr ansposition is a rare form of congenital heart disease, although survi val into adult life may be expected. Little information is available o n the long-term prognosis of these patients once they have reached adu lthood. This study focuses exclusively on patients >18 years old follo wed up al a single tertiary referral center. Methods. The charts of al l patients with a diagnosis of congenitally corrected transposition of the great arteries from the Toronto Congenital Cardiac Centre for Adu lts since 1985 were reviewed, Data were available for 52 patients, 26 of whom had undergone radionuclide angiography, Mortality, clinical an d functional status, surgical procedures and complications were review ed. Results. Thirteen patients (25%) died; age at death was 38.5 +/- 1 2.5 years (mean +/- SD). The current age of survivors is 32.7 years (r ange 18.2 to 54.3), Of the survivors, 17 had palliative procedures, an d 25 had definitive repair, 11 of whom required reoperation. Left vent ricle to pulmonary artery conduit replacement was necessary in seven p atients. Eighteen patients have permanent pacemakers, nine of whom dev eloped complete heart block perioperatively, Nine patients developed p rogressive atrioventricular (AV) block unrelated to operation. Suprave ntricular arrhythmias occurred in 15 patients, Progressive systemic AV valve regurgitation developed in 10 patients and endocarditis in 6. C onclusions. Congenitally corrected transposition in the adult patient is not a benign condition. Late complications are common and warrant c areful, long term follow-up.