Aetiology and 30-Year Long-Term Outcome of Children with Cardiomyopathy Necessitating Heart Transplantation
Open Access
- 27 November 2020
- journal article
- research article
- Published by MDPI AG in Journal of Personalized Medicine
- Vol. 10 (4), 251
- https://doi.org/10.3390/jpm10040251
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.This publication has 37 references indexed in Scilit:
- Cardiac Disorders and Pathophysiology of Sarcomeric ProteinsPhysiological Reviews, 2019
- Pediatric CardiomyopathiesCirculation Research, 2017
- Cardiac Manifestations and Associations with Gene Mutations in Patients Diagnosed with RASopathiesPediatric Cardiology, 2016
- MYH7-related myopathies: clinical, histopathological and imaging findings in a cohort of Italian patientsOrphanet Journal of Rare Diseases, 2016
- Late outcome after paediatric heart transplantation in FinlandInteractive CardioVascular and Thoracic Surgery, 2016
- Lessons learned from the Pediatric Cardiomyopathy Registry (PCMR) Study GroupCardiology in the Young, 2015
- Cardiomyopathy Phenotypes and Outcomes for Children With Left Ventricular Myocardial Noncompaction: Results From the Pediatric Cardiomyopathy RegistryJournal of Cardiac Failure, 2015
- Pediatric heart transplantation: 23-year single-center experienceEuropean Journal of Cardio-Thoracic Surgery, 2011
- The Epidemiology of Childhood Cardiomyopathy in AustraliaThe New England Journal of Medicine, 2003
- The Incidence of Pediatric Cardiomyopathy in Two Regions of the United StatesThe New England Journal of Medicine, 2003