Yuli Kim, MD

Risk assessment of adults with congenital heart disease for untoward outcomes from SARS-CoV-2 infection has been hampered by lack of data on this particular form of heart disease. Early studies during the COVID-19 pandemic as well as subsequent meta-analyses have identified cardiac disease as a risk factor for severe illness and mortality but are specific to acquired forms of heart disease such as atherosclerosis and HF (Yang J, et al. Int J Infect Dis. 2020;doi:10.1016/j.ijid.2020.03.017; Rosenthal N, et al. JAMA Netw Open. 2020;doi: 10.1001/jamanetworkopen.2020.29058). This has left clinicians who care for the adult congenital heart disease population reliant on clinical judgement for management and counseling and the patients themselves unsure as to where they fall on the spectrum of risk.

Schwerzmann and colleagues have provided some insight into this very simple question — among those with adult congenital heart disease, who has gotten ill and/or died from COVID-19? In this multicenter study of adults with congenital heart disease followed in specialty clinics across Europe, investigators describe 105 patients who were infected with SARS-CoV-2 to answer just this question. Characteristics associated with a complicated disease course (including death) in 13 patients included older age, greater than two medical comorbidities (ie, hypertension, diabetes, atherosclerosis, etc), obesity and cyanosis.

In some ways, these findings were both expected and surprising. Given the youthful age of this cohort, it was notable that age fell out of the multivariable model, leaving medical comorbidities and obesity as predictors of severe illness, similar to the non-adult congenital heart disease population. What is surprising is the lack of association between severe illness and disease complexity; for example, those with Fontan physiology or systemic right ventricles did not necessarily do worse, which is somewhat contrary to clinical judgement. The numbers are small, and it would be premature to state that there is no relationship between underlying congenital heart defect and poor outcome in COVID-19, but this is in line with a recent international multicenter study that came to the same conclusion (Broberg CS, et al. J Am Coll Cardiol. 2021;doi:10.1016/j.jacc.2021.02.023). In fact, cyanosis (which includes unrepaired cyanotic patients and those with Eisenmenger syndrome) was the only congenital heart disease-specific characteristic associated with a complicated disease course, and notably also found in the same international study.

To the provider, the take-home here is congenital heart disease complexity is not the driver of risk except for those who are cyanotic. Like the general population, medical comorbidities merit attention in an assessment of the patient as a whole and are another reason to emphasize lifestyle and prevention in longitudinal care. To the adult with congenital heart disease, the results of this study can be reassuring to many young, healthy adults with congenital heart disease who have struggled to understand where they “fit” in the picture of the COVID-19 pandemic. Clearly, more studies dedicated to the impact of SARS-CoV-2 on this complex cardiac population are warranted, but this is a start.