Dilated cardiomyopathy in a national paediatric population

Muhammad Bassem Jammal Addin, David Young, Sarah McCarrison, Lindsey Hunter

Research output: Contribution to journalArticle

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.

LanguageEnglish
Pages1229-1235
Number of pages7
JournalEuropean Journal of Pediatrics
Volume178
Issue number8
Early online date11 Jun 2019
DOIs
Publication statusPublished - 31 Aug 2019

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Dilated Cardiomyopathy
Pediatrics
Population
Mortality
Morbidity
Cardiomyopathies
Inborn Genetic Diseases
Vitamin D Deficiency
Virus Diseases
Survival Rate

Keywords

  • dilated cardiomyopathy
  • Myocarditis
  • vitamin D deficiency

Cite this

Jammal Addin, Muhammad Bassem ; Young, David ; McCarrison, Sarah ; Hunter, Lindsey. / Dilated cardiomyopathy in a national paediatric population. In: European Journal of Pediatrics. 2019 ; Vol. 178, No. 8. pp. 1229-1235.
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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.",
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Dilated cardiomyopathy in a national paediatric population. / Jammal Addin, Muhammad Bassem; Young, David; McCarrison, Sarah; Hunter, Lindsey.

In: European Journal of Pediatrics, Vol. 178, No. 8, 31.08.2019, p. 1229-1235.

Research output: Contribution to journalArticle

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N2 - 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.

AB - 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.

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