Background: Estimates of long-term survival are required to adequately assess the variety of health and social services required by those with congenital heart disease (CHD) throughout their lives.
Methods and results: Medline, Embase, and Scopus were searched from inception to June 2015 using MeSH headings and keywords. Population-based studies that ascertained all persons born with CHD within a predefined area and reported survival estimates at ≥5 years were included. Unadjusted survival estimates for each CHD subtype at ages 1 year, 5 years, 10 years, and so forth were extracted. Pooled survival estimates for each age were calculated using meta-analyses. Metaregression was performed to examine the impact of study period on survival. Of 7840 identified articles, 16 met the inclusion criteria. Among those with CHD, pooled 1-year survival was 87.0% (95% CI 82.1-91.2), pooled 5-year survival was 85.4% (95% CI 79.4-90.5), and pooled 10-year survival was 81.4% (95% CI 73.8-87.9). There was significant heterogeneity of survival estimates among articles (P<0.001 for 1-, 5-, and 10-year survival). A more recent study period was significantly associated with greater survival at ages 1 year (P=0.047), 5 years (P=0.013), and 10 years (P=0.046). Survival varied by CHD subtype, with 5-year survival being greatest for those with ventricular septal defect (96.3%, 95% CI 93.7-98.2) and lowest for those with hypoplastic left heart (12.5%, 95% CI 0.0-41.4).
Conclusions: Among persons with CHD, the mortality rate is greatest during the first year of life; however, this systematic review and meta-analysis showed that survival decreases gradually after infancy and into adulthood.
Keywords: congenital; heart defects; survival.
© 2016 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.