Abstract
Paediatric cardiomyopathies are a heterogenous group of rare disorders, characterised by mechanical and electrical abnormalities of the heart muscle. The overall annual incidence of childhood cardiomyopathies is estimated at about 1 per 100,000 children and is significantly higher during the first 2 years of life. Dilated cardiomyopathies account for approximately half of the cases. Hypertrophic cardiomyopathies form the second largest group, followed by the less common left ventricular non-compaction and restrictive phenotypes. Infectious, metabolic, genetic, and syndromic conditions account for the majority of cases. Congestive heart failure is the typical manifestation in children with dilated cardiomyopathy, whereas presenting symptoms are more variable in other phenotypes. The natural history is largely influenced by the type of cardiomyopathy and its underlying aetiology. Results from a national population-based study revealed 10-year transplant-free survival rates of 80, 62, and 48% for hypertrophic, dilated and left ventricular non-compaction cardiomyopathies, respectively. Long-term survival rates of children with a restrictive phenotype have largely been obscured by early listing for heart transplantation. In general, the majority of adverse events, including death and heart transplantation, occur during the first 2 years after the initial presentation. This review provides an overview of childhood cardiomyopathies with a focus on epidemiology, natural history, and outcomes.
Highlights
Paediatric cardiomyopathies form an uncommon and heterogenous group of disorders, which are characterised by structural, mechanical, and electrical abnormalities of the heart muscle [1, 2]
The European Society of Cardiology classifies cardiomyopathies according to their predominant phenotype, but does not recognise left ventricular non-compaction as a separate entity, whereas the American Heart Association classifies cardiomyopathies according to their aetiology, with left ventricular non-compaction considered to be a separate entity [25, 26]
This review summarises the most common forms of paediatric cardiomyopathies, with a focus on epidemiology and natural history
Summary
Paediatric cardiomyopathies form an uncommon and heterogenous group of disorders, which are characterised by structural, mechanical, and electrical abnormalities of the heart muscle [1, 2]. DCM is the most common type of childhood cardiomyopathy It comprised about half of all cases in the Paediatric Cardiomyopathy Registry (PCMR), a large multicentre North American study, as well as in the National Australian Childhood Cardiomyopathy Study (NACCS), a population-based cohort study which included children younger than 10 years of age at diagnosis. A nationwide cardiomyopathy study from Finland, which included only idiopathic cardiomyopathy cases, demonstrated an overall DCM incidence of 0.34 per 100,000 children up to 20 years and an 11-fold higher incidence during the first year of life (see Table 1) [18]. Data from the PCMR and NACCS demonstrated an overall 5 year transplant-free survival of 54–65 % (see Table 2) [20, 21]. IEM, inborn errors of metabolism; MFS, malformation syndromes; NMD,neuromuscular disorders; FRDA, Friedreich’s ataxia. aNumbers represent overall survival rates by aetiologies, transplantation status not further specified
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