Risk of heart failure-related death and transplant after congenital heart surgery in childhood Open Access

Wright, Lydia (Spring 2020)

Permanent URL: https://etd.library.emory.edu/concern/etds/5712m766p?locale=en



Congenital heart disease (CHD) is a major cause of heart failure (HF) in children and young adults, but the longitudinal risk of HF-related death or transplantation after congenital heart surgery (CHS) is unknown. We aimed to describe this risk and how it varies across the spectrum of CHD.

Methods and Results

We performed a retrospective cohort study of all patients in the Pediatric Cardiac Care Consortium, a US-based multi-center registry of pediatric cardiac surgery, who were discharged transplant-free after their first CHS between 1983 and 2003. Outcomes were obtained by linkage to national death and transplant databases; primary outcome was time from CHS discharge to HF death, transplantation, or ventricular assist device placement. Non-HF death was accounted for as a competing risk. Primary outcome rates were compared to the general population. Among 35,611 patients who survived their first CHS, there were 715 HF-related events over 33 years of follow-up (median 18; IQR 14-22). The cumulative incidence of HF events at 20 years of follow-up was 2.1% (95% CI: 1.9%-2.2%). After risk factor adjustment, hazard ratio (HR) for HF events compared to mild CHD was 30 (95% CI 20-44) for single ventricle (1V), 10 (95% CI 7-14) for severe two-ventricle (2V), and 3.2 (95% CI 2.2-4.6) for moderate CHD. In addition, the presence of a systemic right ventricle was associated with a higher risk of HF-related events in those with severe disease (2V and 1V). All groups had higher rates of HF-related death than the general population.


In this large US cohort, risk of HF-related events after CHS is high across the (entire) spectrum of CHD. While those with single or systemic right ventricles are at highest risk, even those with mild disease are at greater risk than the general population.

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