Abstract
Studies assessing the long-term outcome after heart transplantation HTX in patients with cardiomyopathy (CM) in the paediatric age range are rare. The aim of this study was to determine the survival rate of children with CM undergoing HTX and to analyse how aetiology of cardiomyopathy influenced morbidity and mortality. We retrospectively analysed the medical records of children; who were transplanted in our centre between June 1988 and October 2019. 236 heart transplantations were performed since 1988 (9 re-transplants). 98 of 227 patients (43.2%) were transplanted because of CM. Survival rates were 93% after 1; 84% after 10 and 75% after 30 years. Overall; the aetiology of CM could be clearly identified in 37 subjects (37.7%). This rate increased up to 66.6% (12/19) by applying a comprehensive diagnostic workup since 2016. The survival rate was lower (p < 0.05) and neurocognitive deficits were more frequent (p = 0.001) in subjects with systemic diseases than in individuals with cardiac-specific conditions. These data indicate that the long-term survival rate of children with CM after HTX in experienced centers is high. A comprehensive diagnostic workup allows unraveling the basic defect in the majority of patients with CM undergoing HTX. Aetiology of CM affects morbidity and mortality in subjects necessitating HTX.
Highlights
Heart transplantation (HTX) is the final therapeutic option for children with terminal heart failure
The following items were extracted from the medical records: type of CM (DCM, hypertrophic CM (HCM), restrictive CM (RCM), non-classifiable CM, arrhythmogenic right ventricular CM (ARVC)), age at transplantation, age at death, cause of CM, all information about diagnostics performed to elucidate the basic defect of CM and potential co-morbidities such as cognitive or neurological deficits
Age at HTX was 12 years in 26 (26.5%). 70 (71.4%) subjects suffered from DCM, 10 (10.4%) from RCM, 12 (12.2%) from non-compaction CM (NCM), 5 (5%) from HCM, and one from ARVC (1%)
Summary
Heart transplantation (HTX) is the final therapeutic option for children with terminal heart failure. Besides severe congenital heart defects (CHD), cardiomyopathy (CM) represents an increasing indication for HTX. CM is defined as a cardiac dysfunction based on electrical or mechanical malfunction in the absence of CHD or abnormal loading condition [1]. The incidence of CM in the pediatric age range is 1.1–1.5 per 100,000 [2,3]. Viral infections (myocarditis), defects of cardiac-specific proteins (e.g., MYH7, MYBPC3), systemic metabolic or neurodegenerative disorders (e.g., Pompe disease, Friedreich ataxia) and systemic genetic conditions (e.g., RASopathies) are common causes [7,8,9]
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