The highest mortality rates in childhood dilated cardiomyopathy occur during the first year after diagnosis

• 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.

Ämnesord

MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Pediatrik (hsv//swe)

MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Pediatrics (hsv//eng)

Nyckelord

Cardiomyopathy

cohort study

dilated cardiomyopathy

incidence mortality

Publikations- och innehållstyp

ref (ämneskategori)

art (ämneskategori)