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The highest mortality rates in childhood dilated cardiomyopathy occur during the first year after diagnosis
Department of Paediatrics, Örebro University Hospital, Örebro, Sweden.
The Queen Silvia Children`s Hospital, Sahlgrenska University Hospital, Institution of Clinical Sciences, Gothenburg University, Sweden.
Örebro University, School of Medical Sciences. Clinical Epidemiology and Biostatistics.ORCID iD: 0000-0002-3649-2639
Örebro University, School of Medical Sciences. Örebro University Hospital. Clinical Epidemiology and Biostatistics, School of Medical Sciences, Örebro University, Örebro, Sweden; Unit of Biostatistics, Institute of Environmental Medicine, Karolinska Institutet, Stockholm, Sweden.ORCID iD: 0000-0002-3552-9153
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2018 (English)In: Acta Paediatrica, ISSN 0803-5253, E-ISSN 1651-2227, Vol. 107, no 4, p. 672-677Article in journal (Refereed) Published
Abstract [en]

AIM: The aim of the study was to assess the incidence, mortality and morbidity of dilated cardiomyopathy (DCM) and non-compaction of the left ventricle (LVNC) in Swedish children.

METHODS: We reviewed hospital records of all children with dilated cardiomyopathy (DCM) or left ventricular non-compaction 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 non-compaction 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.

Place, publisher, year, edition, pages
John Wiley & Sons, 2018. Vol. 107, no 4, p. 672-677
Keywords [en]
Cardiomyopathy, cohort study, dilated cardiomyopathy, incidence mortality
National Category
Pediatrics
Identifiers
URN: urn:nbn:se:oru:diva-63447DOI: 10.1111/apa.14183ISI: 000427002500023PubMedID: 29224255Scopus ID: 2-s2.0-85038892042OAI: oai:DiVA.org:oru-63447DiVA, id: diva2:1167874
Note

Funding Agencies:

Örebro County Research Committee  OLL-524371 

Sahlgrenska University Hospital  

Available from: 2017-12-19 Created: 2017-12-19 Last updated: 2018-05-08Bibliographically approved

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Fall, KatjaCao, Yang

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