Cardiac transplantation in adults with congenital heart disease

Abstract

Objectives: Advances in surgical and medical management of congenital heart disease result in survival of an increasing number of grown-up congenital heart (GUCH) patients. As myocardial dysfunction can occur, some of them require heart transplantation (HTx). Methods and results: Fourteen GUCH patients (mean age 30.6, range 16–58 years) underwent HTx between April 1986 and June 2005 at our institution. The diagnoses were: single ventricle (n=4), d-TGA and previous atrial switch operation (n=2), l-TGA (n=1), Morbus Uhl (n=3), others with VSD and complex valve surgery. Refractory heart failure (NYHA IV) was present in all. Four patients received heart transplants after mechanical circulatory support (3 ECMO, 1 BVAD Berlin Heart). Nine patients had a mean of 2 previous cardiac operations. There was 1 early death due to infection. The Kaplan-Meier survival rate was 93% at 1 year, 84% at 5 and 84% at 10 years. No anatomic or surgical risk factor was predictive of death. Outcome was compared with matched (age, gender, time of HTx) controls consisting of 70 patients with dilated cardiomyopathy (DCM) and 28 with ischemic heart disease (IHD). Survival rates of the control groups did not differ significantly from those of GUCH (1 year, 5 years, 10 years: DCM 92%, 88%, 84%; IHD 9%, 89%, 75%). Conclusions: Successful HTx can be achieved in patients with GUCH disease, with an outcome similar to that of patients with acquired heart disease. Detailed assessment of cardiac anatomy and careful surgical planning are essential to obtain optimal results.