Key result
The combined incidence of DCM and LVNC in Swedish children was 0.77 per 100,000 person-years (95% CI 0.59-0.96), with a 30% risk of death or transplant and 21% mortality in the first year.
Why the study?
What are the incidence, mortality, and morbidity rates of dilated cardiomyopathy and left ventricular noncompaction in children?
Cohort (n=69)
Yes
What are the incidence, mortality, and morbidity rates of dilated cardiomyopathy and left ventricular noncompaction in children?
Childhood DCM and LVNC have a combined incidence of 0.77 per 100,000 person-years, with the highest mortality risk occurring during the first year after diagnosis.
High early mortality in childhood DCM/LVNC warrants close initial monitoring; leaves open etiology-specific outcomes for prospective studies.
AIM: The aim of the study was to assess the incidence, mortality and morbidity of dilated cardiomyopathy (DCM) and noncompaction of the left ventricle (LVNC) in Swedish children. METHODS: We reviewed hospital records of all children with dilated cardiomyopathy (DCM) or left ventricular noncompaction cardiomyopathy (LVNC) up to the age of 18 in the healthcare region of western Sweden from 1991 to 2015. RESULTS: In total, 69 cases (61% males) were identified. The combined incidence of DCM and LVNC was 0.77 (95% CI 0.59-0.96) per 100 000 person years. Children were divided into six groups, and their outcomes were analysed depending on their aetiology. Idiopathic DCM was reported in 43%, and familial dilated and left ventricular noncompaction aetiology was present in 32%. DCM due to various diseases occurred in 8%. DCM associated with neuromuscular diseases was present in 16%. The overall risk of death or receiving transplants in children with idiopathic and familial DCM was 30% over the study period, and 21% died in the first year after diagnosis. CONCLUSION: The combined incidence of DCM and LVNC was similar to previous reports. Most children with idiopathic DCM presented during infancy, and mortality was highest during the first year after diagnosis.
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Fadl et al. (2017) conducted a cohort in Dilated cardiomyopathy (DCM) and left ventricular noncompaction cardiomyopathy (LVNC) (n=69). Dilated cardiomyopathy and left ventricular noncompaction was evaluated on Combined incidence of DCM and LVNC per 100,000 person years (95% CI 0.59-0.96). The combined incidence of DCM and LVNC in Swedish children was 0.77 per 100,000 person-years (95% CI 0.59-0.96), with a 30% risk of death or transplant and 21% mortality in the first year.
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