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Dilated cardiomyopathy in a national paediatric population

  • Muhammad Bassem Jammal AddinEmail author
  • David Young
  • Sarah McCarrison
  • Lindsey Hunter
Original Article

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.

Keywords

Dilated cardiomyopathy Myocarditis Vitamin D deficiency 

Abbreviations

ECLS

Extra-corporeal life support

DCM

Dilated cardiomyopathy

FS

Fractional shortening

MPS

Mucopolysaccharidosis

PCR

Polymerase chain reaction

SCD

Sudden cardiac death

SIMD

Scottish Index of Multiple Deprivation

Notes

Authors’ Contributions

Dr. Jammal Addin conceptualized and designed the study, drafted the manuscript, interpreted the data and completed all subsequent revisions until submission; Dr. Young carried out the statistical analysis and critically reviewed the manuscript; Dr. McCarrison assisted with drafting the manuscript, the design and conduct of the study; Dr. Hunter conceptualized and designed the study, advised in presentation of analysis results, and revised the drafts critically for important intellectual content. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

Compliance with ethical statements

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Paediatric CardiologyUniversity Hospitals Bristol NHS Foundation TrustBristolUK
  2. 2.Department of Mathematics & StatisticsStrathclyde UniversityGlasgowUK
  3. 3.Department of Paediatric CardiologyRoyal Hospital for ChildrenGlasgowUK

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