"Reader's Digest": Hot Topics in Congenital and Pediatric Cardiology in the Year 2020

Quick Takes

  • Adult-onset cardiovascular disease is a risk factor for worse outcomes in patients with COVID-19. However, CHD patients without concomitant genetic syndromes, and those who are physiologically well-compensated do not appear to be disproportionately impacted by this disease.
  • MIS-C can lead to serious and life-threatening illness in previously healthy children and adolescents. Left ventricular dysfunction is the most common cardiac manifestation followed by coronary artery involvement.
  • Patients with HLHS and low socioeconomic status have worse neurodevelopmental and functional status outcomes at age 6 years.

Twenty-twenty was the year of the Coronavirus disease  (COVID-19) pandemic as well as emerging awareness for the social determinants of health. With these in mind, three articles with these themes are highlighted below. These address: (1) the impact of COVID-19 in individuals with congenital heart disease (CHD); (2) the temporal relationship of multisystem inflammatory syndrome in children (MIS-C) to the COVID-19 pandemic in the United States; (3) the association  of lower socioeconomic status to worse long-term neurodevelopmental outcomes in children with hypoplastic left heart syndrome (HLHS).

The Impact of COVID‐19 on Patients with CHD Across the Lifespan: The Experience of a Large Center in New York City1

This study assessed the impact and predictors of COVID‐19 infection and severity in a cohort of CHD patients  between March 1 and July 1, 2020, with the primary endpoint being death or the need for hospitalization and/or respiratory support. Fifty-three patients met inclusion criteria, with a median age of 34 years, 10 (19%) were <18 years old and 52 were symptomatic. Thirty-one (58%) had complex congenital anatomy including 10 (19%) with Fontan palliation. Eight patients (15%) had a genetic syndrome including five (9%) with Trisomy 21, two (4%) with DiGeorge syndrome and one (2%) with VACTERL association. Among adults, 18 (41%) were physiologic class C or D. For the entire cohort, nine (17%) had moderate/severe infection, including three deaths (6%). Both patients living in long-term care facilities at the time of infection died. Of note, six of the seven hospitalized patients with moderate/severe infection had worsening hypoxemia requiring supplemental oxygen and three required intubation. One hospitalized patient required inotropic support. Two underwent transthoracic echocardiography during admission with no change from baseline. After correcting for multiple comparisons, the presence of a genetic syndrome (OR=35.82: p=0.0002), and in adults, physiological Stage C or D (OR=19.38: p=0.002), were significantly associated with worse outcomes. The study concluded that despite evidence that adult-onset cardiovascular disease is a risk factor for worse outcomes among patients with COVID-19, patients with CHD without concomitant genetic syndrome, and adults who are not at advanced physiological stage, do not appear to be disproportionately impacted.

The Multisystem Inflammatory Syndrome in Children (MIS-C) in the United States2

In late April 2020, reports emerged from the United Kingdom of children presenting with fever and hyperinflammation temporally associated with COVID-19 and with clinical features similar to incomplete Kawasaki disease (KD) or toxic shock syndrome,2 named MIS-C by the Centers for Disease Control (CDC). This study investigated the epidemiology and clinical course of MIS-C in 53 centers in the United States from March 15 to May 20, 2020. Patients <21 years of age with fever >24 hours, laboratory evidence of inflammation, multi-organ involvement, and who had severe acute respiratory syndrome coronavirus 2 (SARS-COV-2) infection were included. There were 186 patients from 26 states with a median age of 8.3 years. Most patients (70%) were positive for SARS-CoV-2 based on PCR or antibody testing, with the remainder having exposure to others with COVID-19. Gastrointestinal organ system involvement was most common (92%), followed by cardiovascular (80%), hematologic (76%), mucocutaneous (74%), and respiratory (70%) involvement. The median duration of hospitalization was 7 days. Two percent died, with 80% requiring intensive care, and 20% and 48% requiring mechanical ventilation and vasoactive support, respectively. Ventricular systolic dysfunction was commonly seen, which was severe in 5% of patients. Coronary artery involvement (defined as coronary dimensions with z-scores ≥2.5) were seen in 8% of patients. Intravenous immune globulin was used in 77% of patients, glucocorticoids in 49%, and interleukin-6 or 1RA inhibitors in 20%. The authors concluded that MIS-C led to serious and life-threatening illness in previously healthy children and adolescents. Left ventricular dysfunction is the most common cardiac manifestation of MIS-C, followed by coronary artery involvement. Hemodynamic support and immunomodulatory therapies are primary treatments. Most children recover, but medium- and long-term sequelae are unknown.

Socioeconomic Status and Long-term Outcomes in Single Ventricle Heart Disease3

Low socioeconomic status (SES) is emerging as an important risk factor for higher short-term mortality in HLHS. However, little is known about how SES affects outcomes over the long-term. This study linked data from the Single Ventricle Reconstruction trial which followed patients at least until the age of 6 years to US Census Bureau data. The goal is to analyze the relationship of neighborhood SES tertiles with mortality and need for transplantation, neurodevelopment, quality of life, and functional status at 5- and 6-years post-Norwood procedure (N = 525). Scores were derived from six census-block group measures related to wealth and income, education, and occupation, with higher scores indicating higher neighborhood SES. Patients were divided into SES tertiles. Patients in the lowest SES tertile were more likely to be racial minorities, older at stage 2 and Fontan procedures, and to have more complications and fewer cardiac catheterizations at follow-up (all P <.05) compared with patients in higher SES tertiles. Unadjusted mortality was highest for patients in the lowest SES tertile and lowest in the highest tertile (41% vs. 29%, respectively; P = .027). Adjustment for patient birth and Norwood factors slightly attenuated these differences (P = .055). Patients in the lowest SES tertile reported lower functional status and lower fine motor, problem-solving, adaptive behavior, and communication skills at 6 years (all P <.05). These differences persisted after adjustment for baseline and post-Norwood factors. Quality of life did not differ by SES. The study concluded that patients with HLHS with low SES have worse neurodevelopmental and functional status outcomes at 6 years. Future investigations into modifiable risk factors for neurodevelopmental outcomes are needed to better understand areas of intervention and improve outcomes across the spectrum of SES.


  1. Lewis MJ, Anderson BR, Fremed M, et al. Impact of coronavirus disease 2019 (COVID-19) on patients with congenital heart disease across the lifespan: the experience of an academic congenital heart disease center in New York City. J Am Heart Assoc 2020;9:e017580.
  2. Feldstein LR, Rose EB, Horwitz SM, et al. Multisystem inflammatory syndrome in U.S. children and adolescents. N Engl J Med 2020; 383:334-46.
  3. Bucholz EM, Sleeper LA, Goldberg CS, et al. Socioeconomic status and long-term outcomes in single ventricle heart disease. Pediatrics 2020;146:e20201240.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Diabetes and Cardiometabolic Disease, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, CHD and Pediatrics and Quality Improvement, Heart Failure and Cardiac Biomarkers, Interventions and Imaging, Interventions and Structural Heart Disease, Echocardiography/Ultrasound

Keywords: COVID-19, Coronavirus, severe acute respiratory syndrome coronavirus 2, Fontan Procedure, Quality of Life, Immunoglobulins, Intravenous, Glucocorticoids, Receptors, Interleukin, Interleukin-6, Mucocutaneous Lymph Node Syndrome, Hypoplastic Left Heart Syndrome, DiGeorge Syndrome, Cardiovascular Diseases, Respiration, Artificial, Coronary Vessels, Shock, Septic, Long-Term Care, Social Determinants of Health, Heart Defects, Congenital, Social Class, Echocardiography, Adaptation, Psychological, Risk Factors, Hospitalization, Ventricular Dysfunction, Left, Cardiac Catheterization, Critical Care, Hemodynamics, Centers for Disease Control and Prevention (U.S.), Immunomodulation, Polymerase Chain Reaction, Problem Solving, Intubation, Intratracheal, Oxygen

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