Heart-lung and lung transplantation in grown-up congenital heart disease: long-term single centre experience.

Objective: Because of considerable progress in paediatric cardiac surgery life expectancy of patients with congenital heart disease (CHD) has improved significantly over the years. There are a growing number of adults with CHD presenting with progressive decline of cardiopulmonary function and Eisenmenger's syndrome. We analysed our experience with heart-lung and lung transplantation in this patient group.

Methods: Since 1988, a total of 46 heart-lung transplantations and 5 double lung transplantations have been performed in adults with CHD at our institution. Underlying diagnoses were: ventricular septal defect, atrial septal defect, persistent ductus arteriosus and others. Pulmonary hypertension was present in all patients. Twelve patients had undergone previous cardiac procedures. All patients were included in this retrospective analysis. Mean follow up was 5.1+/-4.7 years. Patient survival was estimated with the Kaplan-Meier method and analysed using the log-rank test.

Results: Thirty-day mortality was 11.8% (n=6). Survival was 80% at 1 year, 69% at 5 years and 53% at 10 years. Major causes of death were infection and sepsis, chronic rejection, initial graft failure and acute rejection. Compared to the overall mortality after lung and heart-lung transplantation for other indications at our institution there was no significant difference (1 year, 5 years, 10 years: 76%; 60%; 45%), but a tendency towards a better long-time survival of the CHD patients.

Conclusions: Lung and heart-lung transplantation can be performed with an acceptable risk and a favourable long-term outcome in patients with grown-up CHD. Careful patient selection and planning of the surgical strategy is essential in this high-risk patient population.