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.
| Language | English |
|---|---|
| Pages | 1229-1235 |
| Number of pages | 7 |
| Journal | European Journal of Pediatrics |
| Volume | 178 |
| Issue number | 8 |
| Early online date | 11 Jun 2019 |
| DOIs | |
| Publication status | Published - 31 Aug 2019 |
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Keywords
- dilated cardiomyopathy
- Myocarditis
- vitamin D deficiency
Cite this
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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 journal › Article
TY - JOUR
T1 - Dilated cardiomyopathy in a national paediatric population
AU - Jammal Addin, Muhammad Bassem
AU - Young, David
AU - McCarrison, Sarah
AU - Hunter, Lindsey
N1 - This is a post-peer-review, pre-copyedit version of an article published in European Journal of Pediatrics. The final version is available online at: https://doi.org/10.1007/s00431-019-03404-w
PY - 2019/8/31
Y1 - 2019/8/31
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.
KW - dilated cardiomyopathy
KW - Myocarditis
KW - vitamin D deficiency
U2 - 10.1007/s00431-019-03404-w
DO - 10.1007/s00431-019-03404-w
M3 - Article
VL - 178
SP - 1229
EP - 1235
JO - European Journal of Pediatrics
T2 - European Journal of Pediatrics
JF - European Journal of Pediatrics
SN - 0340-6199
IS - 8
ER -