Progressive tricuspid valve disease in patients with congenitally corrected transposition of the great arteries

Background-The outcome of patients with corrected transposition of the great arteries (CTGA) is variably affected by associated intracardiac defects, tricuspid valve competence, and systemic right ventricular (RV) function. The relative importance of these factors in long-term outcome has not been evaluated. Methods and Results-Since 1958, 40 patients with CTGA were studied to determine risk factors for poor outcome, including age, open heart surgery, tricuspid insufficiency (TI), cardiac rhythm, pulmonary overcirculation, and RV dysfunction. Follow-up ranged from 7 to 36 years (mean 20 years). Intracardiac repair was performed in 21 patients; 19 were unoperated or had closed heart procedures. For the purposes of this study the designation TIs refers to at least moderately severe TI as delineated by echocardiogram and/or angiography. TIs was the only independently significant factor for death (P=0.01), and in turn, only the presence of a morphologically abnormal TV predicted TIs (P=0.03), Twenty-year survival without TIs was 93%, but only 49% with TIs. Poor long-term postoperative outcome was due to TIs in all but 1 patient; 20-year survival rates for operated patients with and without TIs were 34% and 90%, respectively (P=0.002). Similarly, 20-year survival rates for unoperated patients with and without TIs were 60% and 100%, respectively, whether or not attempts to repair the TI were made (P=0.08). Conclusions--TIs represents the major risk factor for CTGA patients; RV dysfunction appears to be almost always secondary to long-standing TI. Decisions related to surgical interventions with or without associated lesions must be strongly influenced by the status of the tricuspid valve.