Postsurgical course of patients with congenitally corrected transposition of the great arteries

Because congenitally corrected transposition is a rare congenital anomaly, little is known about the prognosis of patients with this syndrome. The pre sent study evaluated the functional status and clinical course of 73 patien ts (42 male and 31 female) aged 10 days to 73 years. Follow-vp was 1 week t o 37 years (mean 12.7 years), Particular attention was paid to the systemic right ventricle, considering the current controversies about long-term rig ht ventricular function. Survival of patients with this condition in genera l was significantly below normal, The total mortality rate was 11% (8 patie nts) after a mean follow-up of 12.7 years (range 10 days to 37 years). Mean age at death was 18.5 years (range 6 days to 63 years). The mean age of th e survivors at latest follow-up was 21.4 years (range 4 months to 73 years) , In most patients, right ventricular function deteriorated and tricuspid v alve regurgitation increased, which began at a very young age, Patients wit hout associated lesions developed complications at a higher age compared wi th the total group, Right ventricular and tricuspid valvular function deter iorated more frequency in patients following intracardiac operation (28% an d 52%, respectively) compared with patients undergoing palliative intervent ion (16%) or no surgery at all (28%). We conclude that survival of patients with this condition is substantially reduced compared with the natural his tory of an age- and gender-matched general population. This could be explai ned by the associated cardiac defects, but also by the anatomic condition i tself. (C) 1999 by Excerpta Medico, Inc.