Outcome after orthotopic cardiac transplantation in adults with congenitalheart disease

Background-Advances in surgical and medical management have greatly improve d long-term survival rates in patients with congenital heart disease (CHD). As these patients reach adulthood, myocardial dysfunction can occur, lendi ng to cardiac transplantation. Methods and Results We reviewed the pretransplantation and posttransplantat ion courses of 24 patients >18 years old (mean age, 26 years; range, 18 to 56 years) with CHD who received a transplant between January 1985 and Septe mber 1998. The relation between preoperative and perioperative risk factors for complications and death was assessed. Single ventricle was the pretran splantation diagnosis for 12 patients (50%), and d-transposition of the gre at vessels was the diagnosis for 4 patients (16%). Twenty-two patients had a mean of 2 previous operations. At cardiac transplantation, additional sur gical procedures were required to correct extracardiac lesions in 18 patien ts (75%). Refractory heart failure was present in 22 patients, significant cyanosis was present in 7, and protein-losing enteropathy was present in 4. There were 5 early deaths due to bleeding (n=3) and infection (n=2). The K aplan-Meier survival rate after cardiac transplantation was 79% at 1 year a nd 60% at 5 years. No anatomic or surgical Ask factor was predictive of dea th. The outcome of patients with CHD who received a transplant was compared with that for patients without CHD (n=788). Mean bypass and ischemic times were significantly longer in patients with CHD than in patients without CH D. Survival rates after transplantation did not differ significantly betwee n patients with and those without CHD (P=0.83). Conclusions Successful cardiac transplantation is obtainable in adults with complex CHD, with an outcome similar to that of patients without CHD. A de tailed assessment of cardiac anatomy and careful surgical planning ore esse ntial to the pretransplantation and posttransplantation management of these patients.