Methods

Retrospective single-centre analysis of long-term posttransplant outcome, with chart collection of clinical and paraclinical data [this study assessed the long-term outcome of heart (HTx) and heart-lung transplantation (HLTx) in patients with congenital heart disease (CHD) and children with non-congenital cardiac or pulmonary disease.]

Results

From 1984 to 2013, 111 first-HTx, 5 HLTx and 6 re-HTx were performed (62 males), in patients aged 11.7 ± 8.2 y: 96 (79%) aged < 18 y. Cardiopathy included 61 cardiomyopathies (50.8%), 50 CHD (41.7%), 6 retransplants (5%). HLTx included 1 Eisenmenger, 1 PPHT, and 2 pulmonary diseases. Patients with cardiomyopathy were younger than CHD (8.7 y vs. 14.9 y). Seventeen (14%) patients had circulatory mechanical support as bridge to transplant. Acute rejection occurred more frequently within the first year post-transplant or > 5th year in non-compliant teenagers. Overall, 33 patients died (27%), 3.5 ± 4.6 y post-Tx (1 day to 16.4 y, med 1.5 months), due to early multivisceral failure in 6 (18%), pulmonary hypertension in 3 (9%), acute rejection in 7 (21%), graft coronary disease in 6 (18%), sepsis in 5 (15%) and miscellaneous in 6. Graft coronary disease occurred in 15 (12.4%): 4 had re-HTx, 6 died and 5 are alive. Five lymphoma occurred, 4 months to 14 y after HTx, cured in 4 (1 died). Patients survival was 85% at 1 y, 81% at 5 y, 70% at 10 y and 61% at 20 y post-transplant. Graft survival rates were respectively 82%, 68% and 52% at 5 y, 10 y and 20 y post-transplant. Survival did not differ with pretransplant disease, age, gender, pretransplant mechanical support. Mortality was higher in patients with coronary disease (40%) than those free from (25%).

Conclusion

Long-term prognosis after HTx and HLTx is favourable. Graft coronary disease is the main cause of failure, less frequent than in the adult non-CHD heart-transplanted population.