30-Year Long-Term Outcome of Heart Transplanted Children with Cardiomyopathy

Objectives: Early and late mortality have significantly improved during recent decades in pediatric patients after heart transplantation (Htx). The influence of different forms of underlying diseases of cardiomyopathies (CM) has only been evaluated sparsely so far.

Methods: We evaluated our results after Htx in children suffering from cardiomyopathy who have been heart transplanted in the 30 last years.

Results: Over the last three decades 221 children have been transplanted in our heart-center. One of the main group receiving heart transplantation were patients with a CM, in total 89 (in 51 patients reason for CM stayed idiopathic, 19 patients reason for CM was associated with a myocarditis, 17 patients had an underlying disease, and in 2 cases reason for the CM was related to polychemotherapies). The overall survival rate for patients suffering from a cardiomyopathy who underwent Htx was 92, 85, 74, and 74 for 1, 10, 20, and 30 years, respectively.

In the group of multisystemic underlying diseases (e.g. mitochondriopathies four patients, Barth’s syndrome two patients, or LEOPARD syndrome one patient) 4 of 13 patients died, while in the group of cardio specific diseases like sarcomeric disease or myocarditis 23 patients none died, revealing a significant increased mortality in patients with multisystemic underlying diseases (p < 0.05). Next, we examined patients with multisystemic underlying disease for their neurological and cognitive handicaps. In the group with cardio specific disease only one patient had a severe neurological and/or cognitive handicap whereas in the group of multisystemic disorders (n = 13 patients), eight patients had a severe neurological and/or cognitive handicap (p = 0.001) at latest follow-up.

Conclusion: Pediatric HTx is a safe and effective treatment for terminal heart failure in children with cardiomyopathies. Mortality and morbidity of patients with sarcomeric CM or myocarditis differs from morbidity of patients with multisystemic underlying diseases, with a significant higher mortality and morbidity for patients with multisystemic underlying diseases.

No conflict of interest has been declared by the author(s).