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Tuesday, February 07, 2017

Congenital HD survival

Question  Has survivorship in young children with congenital heart disease increased in Sweden?
Finding  A registry-based, prospective, matched-cohort study showed that, among children younger than 5 years, survivorship was increased in those born at the beginning of 1990 compared with children born in the 1970s; however, the overall mortality risk was higher, depending on the complexity of the disease, than in matched controls.
Meaning  Absolute and relative survivorship increased substantially, but mortality remains high in young patients with congenital heart disease compared with matched controls, particularly in subsets of children with complex issues.
Importance  Mortality in patients with congenital heart disease (CHD) has markedly decreased during recent decades because of advancement in pediatric care. However, there are limited data on survival trends in children and young adults with CHD compared with the general population.
Objective  To determine survivorship in children and young adults with CHD compared with matched controls.
Design, Setting, and Participants  A registry-based, prospective, matched-cohort study was conducted in Sweden. Data from the national patient and cause of death registers were linked to identify individuals with CHD born between January 1, 1970, and December 31, 1993, who were registered at or after birth. Follow-up and comorbidity data were collected until December 31, 2011. Survival analyses were performed with the Cox proportional hazards model; these analyses were performed from January 1, 1970, to December 31, 2011. A total of 21 982 patients with CHD in Sweden were identified. The mean (SD) follow-up time was 27.0 (8.86) years. Children serving as controls (n = 219 816) (10 for each patient), matched for birth year, sex, and county, were randomly selected from the general population.
Main Outcomes and Measures  Survivorship in young patients with CHD and controls.
Results  Of the 21 982 patients who were born between 1970 and 1993 and were registered with the diagnosis of CHD, 10 650 were female (48.4%). Median age at index registration was 4.22 years (interquartile range, 17.07 years). Survivorship among children younger than 5 years was increased from 96% in those born in 1970-1979 to 98% in those born in 1990-1993. Hazard ratios (HRs) of death in relation to that in control individuals decreased from 225.84 (95% CI, 136.84-372.70) to 33.47 (95% CI, 22.54-49.70). A substantial, but less pronounced, absolute and relative increase in survivorship was found in older patients (HRs ranged from 24.52; 95% CI, 11.72-51.26, at 5-9 years to 4.27; 95% CI, 2.29-7.95, at 18-29 years). According to a hierarchical CHD classification, the group of patients with the most severe complex defects (ie, common arterial trunk, transposition of the great vessels, double inlet ventricle, hypoplastic left heart syndrome, tetralogy of Fallot, and atrioventricular septal defect) had the highest risk for death (HR, 64.07; 95% CI, 53.39-76.89).
Conclusions and Relevance  Despite substantially increasing absolute and relative survivorship in children and young adults with CHD, the mortality risk remains high compared with the risk in matched controls. Further research on reducing the death rate in this vulnerable group is required.

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