Long-term outcome in congenitally corrected transposition of the great arteries - A multi-institutional study

OBJECTIVES The purpose of this study was to determine long-term outcome in adults with congenitally corrected transposition of the great arteries (CCT GA), with particular emphasis on systemic ventricular dysfunction and conge stive heart failure (CHF). BACKGROUND Patients with CCTGA have the anatomical right ventricle as their systemic pumping chamber, with ventricular dysfunction and CHF being relat ively common in older adults. METHODS Retrospective analysis of records of 182 patients from 19 instituti ons were reviewed to determine current status and possible risk factors for systemic ventricular dysfunction and CHF. Factors considered included age, gender, associated cardiac defects, operative history, heart block, arrhyt hmias and tricuspid (i.e., systemic atrioventricular) regurgitation (TR). RESULTS Both CHF and systemic ventricular dysfunction were common in groups with or without associated cardiac lesions. By age 45, 67% of patients wit h associated lesions had CHF, and 25% of patients without associated lesion s had this complication. The rates of systemic ventricular dysfunction and CHF were higher with increasing age, the presence of significant associated cardiac lesions, history of arrhythmia, pacemaker implantation, prior surg ery of any type, and particularly with tricuspid valvuloplasty or replaceme nt. Aortic regurgitation (a previously unreported problem) was also relativ ely common in this patient population. CONCLUSIONS Patients with CCTGA are increasingly subject to CHF with advanc ing age; this complication is extremely common by the fourth and fifth deca des. Tricuspid (systemic atrioventricular) valvular regurgitation is strong ly associated with RV (anatomical right ventricle connected to aorta in CCT GA patients; systemic ventricle in CCTGA) dysfunction and CHF; whether it i s causative or a secondary complication remains speculative. (C) 2000 by th e American College of Cardiology.