Goldstein, Sarah A, D'Ottavio, Alfred, Spears, Tracy, Chiswell, Karen, Wiedemeier, Manuela, Spates, Toi D, Krasuski, Richard A, Kemper, Alex R, Meyer, Robert E, Hoffman, Timothy M, Walsh, Michael J, Sang, Charlie J, Paolillo, Joseph A, and Li, Jennifer S
Background: Improved pediatric congenital heart disease (CHD) outcomes have led to rapid growth of the adult CHD population. There are few contemporary studies examining causes of death (COD) in adults with CHD. Methods: Patients with CHD with a healthcare encounter between 2008-2013 at one of five tertiary care centers in North Carolina (NC) were identified by ICD-9 code. Those aged ≥ 20 yrs who could be linked to a NC death certificate between 2008-2016 were included. Mean age of death (AOD) & underlying COD were analyzed, with stratification by lesion severity (severe, shunt, valve). Results: A total of 629 adults met inclusion criteria. The mean AOD was 64.2 yrs. Those with severe CHD (n=157, 25%), shunts (n=202, 32%) & valvular lesions (n=174, 28%) had a mean AOD of 46.0, 65.0 & 73.3 yrs, respectively. The most common COD among all adults were cancer (14%), CHD (14%), ischemic heart disease (11%), infection (8%) & lung disease (7%); 43% died from any cardiovascular (CV) disease with 33% of CV deaths due to CHD. The most common COD among those with severe CHD were CHD (41%), cancer (9%) & heart failure (8%); 60% died from CV disease with 68% of CV deaths due to CHD. For those with shunts, the most common COD were ischemic heart disease (15%), cancer (15%) & lung disease (10%); 42% died from CV disease with 21% of CV deaths due to CHD. The most common COD among those with valvular CHD were cancer (17%), ischemic heart disease (11%) & lung disease (9%); 34% died from CV disease with 8% of CV deaths due to CHD. Among those who died during the study period, the odds of death due to CV disease vs. other causes was 2.6 times higher in those with severe CHD compared to non-severe disease (95% CI for OR 1.8-3.2, P<0.0001) and the odds of death due to CHD vs. other causes was 14 times higher in those with severe CHD compared to non-severe disease (95% CI for OR 8.1-23.4, P<0.0001). Conclusions: In those without severe disease, the lifespan of adults with CHD is approaching that of the general population as reported by the CDC. The most common COD differ based on lesion severity, with CHD being most common only among those with severe disease. Among patients with non-severe CHD, the most common COD are similar to the general population. Understanding COD in adults with CHD can lead to improved, targeted preventive care. [ABSTRACT FROM AUTHOR]