Dilated cardiomyopathy in a national paediatric population

Jammal Addin, Muhammad Bassem and Young, David and McCarrison, Sarah and Hunter, Lindsey (2019) Dilated cardiomyopathy in a national paediatric population. European Journal of Pediatrics, 178 (8). pp. 1229-1235. ISSN 1432-1076

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    Abstract

    Dilated cardiomyopathy is the most common form of childhood cardiomyopathy and is known to result in significant morbidity and mortality. This study aims to review the aetiology and associated outcomes of DCM. The median age at diagnosis was 6 months (0–42 months); n = 23 (43.3%) were idiopathic; n = 11 (20.9%) secondary to a viral infection; n = 12 (22.6%) genetic disorders and n = 7 (13.2%) as a result of vitamin D deficiency. There was a significant correlation between aetiology and mortality, r = 0.85, with a lower survival rate in idiopathic and genetic cohorts. Males were significantly less likely to survive to 1 year of age, p = 0.035. The age at diagnosis did not alter survival to 1 year and the predicted survival beyond 1 year was 84.3% (95% CI, 71.3 to 94.5%). Severely impaired left ventricular fractional shortening at presentation (< 15%) was an independent predictor of death, p = 0.002, (95% CI, 11.2 to 14.2%). Conclusion: Paediatric DCM is a heterogeneous disease resulting in significant morbidity. The aetiology alters the age of presentation. Identification of a specific cause is a useful for risk stratification and prognostication. The first year after diagnosis is a critical time period reflected by the significant morbidity and mortality.What is Known:•Paediatric dilated cardiomyopathy (DCM) is the commonest of the childhood cardiomyopathies, with significant associated morbidity and mortality.•DCM is most commonly idiopathic.What is New:•Identifying the aetiology of DCM in the paediatric population aids risk stratification and prognostication.•The first year after diagnosis of DCM is associated with significant mortality.