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Incidence of ESKD and mortality among children with congenital heart disease after cardiac surgery

Parikh CR, Greenberg JH, McArthur E, Thiessen-Philbrook H, Everett AD, Wald R, Zappitelli M, Chanchlani R, Garg AX. Clin J Am Soc Nephrol. 2019; 14(10):1450-7. Epub 2019 Sep 9. DOI: 10.2215/CJN.00690119.


Background and Objectives — Survival after surgical repair for congenital heart disease has markedly improved; however, there are limited data on long-term ESKD and mortality during childhood.

Design, Setting, Participants, and Measurements — We conducted an observational, population-based cohort study of children who had their first surgery for congenital heart disease within 10 years of birth. The study was conducted in Ontario, Canada, where residents have universal access to health care services. Each child who underwent surgical repair was matched to ten children from the general population who were similar in age, sex, index date, rurality, and neighborhood income. Primary outcomes of all-cause mortality and ESKD were reported until March 2015.

Results — We followed 3600 children with congenital heart disease for a median of 5.9 (interquartile range, 2.9–9.0) years after their surgical repair. Median age at first surgery was 150 (interquartile range, 40–252) days and 22% were low birth weight (<2500 g). During follow-up, 140 (4%) children who had surgery for congenital heart disease died and 52 (1%) reached ESKD. The cumulative incidence of death and ESKD at 1, 5, and 10 years was higher in children with surgical repair of congenital heart disease (death: 3%, 4%, and 5%, respectively; ESKD: 1%, 2%, and 2%, respectively) compared with the matched control population without any congenital heart disease (death: 0.06%, 0.10%, and 0.13%, respectively; ESKD: 0.00%, 0.02%, and 0.02%, respectively). The risk of ESKD and death increased with severity of congenital heart disease, with the highest risk in children with hypoplastic left heart syndrome and increased in children who had surgical repair of congenital heart disease compared with those without surgical repair.

Conclusions — The risk of mortality and ESKD is high in children who undergo surgical repair for congenital heart disease compared to the general population.

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