COVID-19 in Congenital Heart Disease: Ten Points to Remember

This is a compilation of key items discussed in the manuscript "Coronavirus Disease 2019 (COVID-19): Pandemic Implications in Pediatric and Adult Congenital Heart Disease".1 This is a rapidly changing field and we hope that you find this summary helpful in managing patients with congenital heart disease infected with COVID-19.

  1. While children initially seem to have milder forms of the illness, the knowledge and experience in caring for patients with COVID-19 are rapidly evolving.
  2. Given the increased risk for severe COVID-19 in adults with underlying cardiac disease, there is concern that patients with congenital heart disease (CHD) may likewise be at increased risk for severe infection, as they are known to have higher risk for complications with viral illnesses including respiratory syncytial virus and influenza.
  3. The coronavirus enters cells through the angiotensin-converting enzyme 2 receptors (ACE-2). ACE-2 are expressed on the surface of epithelial cells within the alveoli and small intestine, which explains the respiratory and gastrointestinal symptoms associated with the disease. ACE converts angiotensin I to angiotensin 2 while ACE-2 deactivates angiotensin 2. Angiotensin 2 stimulates lung injury and ACE-2 serves a role in lung protection as it deactivates angiotensin 2. Viral binding to ACE-2 deactivates the protective role of ACE-2, which may contribute to the severe lung injury seen with COVID-19.
  4. Cardiomyocyte injury appears to be due to an acute and severe inflammatory response akin to a cytokine storm, viral invasion of cardiomyocytes resulting in cellular damage as well as ischemia from severe hypoxia due to acute lung injury.
  5. These groups may be at increased risk for COVID-19: single ventricle patients after Fontan operation, those with chronic cyanosis and depressed ventricular function, individuals with severe pulmonary hypertension, immune-compromised patients (including those who have undergone heart transplantation), infants with unrepaired significant congenital heart disease, and adults with CHD complicated by coronary artery disease or systemic hypertension.
  6. It is recommended that all cardiac medications, including aspirin, anticoagulants, ACE inhibitors, angiotensin receptor blockers, beta blockers, diuretics and antiarrhythmic medications be continued during COVID-19 illness, unless a clear contraindication develops.
  7. The clinician must be cognizant of QT prolonging effects of some of the medications that are used in COVID-19 therapy, including hydroxychloroquine.
  8. Several trials are underway to evaluate the impact of COVID-19 in patients with CHD, their response to therapy, and the efficacy of post exposure prophylaxis.
  9. As cases and clinic visits are postponed or limited, the cardiac care team must evaluate patient care plans and consider the unintended consequence of delayed access to care in these patients.
  10. After manuscript publication, reports of a multisystem inflammatory syndrome in previously well children (MIS-C) temporally associated with COVID-19 infection have emerged.2 MIS-C shares similarities to severe Kawasaki disease (KD), including the development of giant coronary aneurysms, although affected patients tend to be older. At least initially, myocardial involvement appears to be reversible and thought to be related to myocardial stunning and edema rather than inflammatory myocardial injury. Thus, although initial cardiac dysfunction may be severe, most patients seem to recover ventricular function. However, the long-term prognosis of coronary involvement, and whether the response to therapy is similar to KD, remain to be seen.


  1. Alsaied T, Aboulhosn JA, Cotts TB, et al. Coronavirus Disease 2019 (COVID-19): pandemic implications in pediatric and adult congenital heart disease. J Am Heart Assoc 2020. Epub ahead of print.
  2. Belhadjer Z, Meot M, Bajolle F, et al. Acute heart failure in multisystem inflammatory syndrome in children (MIS-C) in the context of global SARS-CoV-2 pandemic. Circulation 2020. Epub ahead of print.

Clinical Topics: Anticoagulation Management, Congenital Heart Disease and Pediatric Cardiology, COVID-19 Hub, Stable Ischemic Heart Disease, Vascular Medicine, Atherosclerotic Disease (CAD/PAD), Congenital Heart Disease, Novel Agents, Statins, Chronic Angina

Keywords: Heart Defects, Congenital, COVID-19, Angiotensin I, Angiotensin Receptor Antagonists, Angiotensin-Converting Enzyme Inhibitors, Coronary Artery Disease, Myocytes, Cardiac, Hydroxychloroquine, Mucocutaneous Lymph Node Syndrome, Coronavirus, Coronavirus Infections, severe acute respiratory syndrome coronavirus 2, Coronary Aneurysm, Aspirin, Diuretics, Respiratory Syncytial Viruses, Anticoagulants

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