"Reader's Digest": Hot Topics in Congenital and Pediatric Cardiology in the Year 2022

Quick Takes

  • Advances in cardiac imaging have improved identification of patients previously thought to be single ventricles who may be candidates for biventricular repair.
  • Improved neuroimaging provides a better understanding of neurodevelopmental outcomes in the congenital heart disease population.
  • COVID-19 vaccine-related myocarditis appears to be a rare self-limiting condition.
  • Two decades after the first transcatheter pulmonary valve replacement procedure, long-term mortality and re-intervention outcomes are comparable to surgical intervention.

The year 2022 was the year of widespread vaccination against Coronavirus disease (COVID-19), resulting in a drastic decrease in the number of cases with less severe infections and hospitalizations. The incidence of multisystem inflammatory syndrome (MIS) in children has also decreased. Concern has been raised regarding vaccination side effects, including myocarditis.

In the field of pediatric cardiology and congenital heart disease (CHD), advances in imaging modalities have improved surgical management and decision making. Collaborative research continues to evaluate outcomes of several cardiac transcatheter devices. The focus on neurodevelopmental outcomes of patients with CHD continues and multifactorial determinants of these outcomes including factors in prenatal life have been identified.

This year also marks the advent of the journal JACC Advances, which will highlight CHD in children and adults, cardio obstetrics, digital health, and other cardiovascular topics. Four articles with these themes are summarized below. These articles address: (1) fetal brain volume as a predictor of neurodevelopmental outcomes in children with CHD; (2) biventricular repair in borderline left hearts: insights from cardiac magnetic resonance (CMR); (3) outcomes after transcatheter pulmonary valve replacement (TPVR), (4) COVID-19 vaccine related myocarditis.

Prenatal Fetal Brain Volume as a Predictor of Neurodevelopmental Outcomes in Children with CHD1
Neurodevelopmental impairment is common in patients with CHD. It is multifactorial and post-natal factors explain only 30% of the variance in outcomes. This study compared prenatal brain magnetic resonance imaging (MRI) and postnatal neurodevelopmental testing at 2 years of 52 patients with isolated CHD and 26 sociodemographically comparable controls. Fetal brain volume predicted 10-21% of the variance in neurodevelopmental outcomes while social class and postnatal factors explained 18-45% of the variance in outcomes. Thus, fetal brain volume may be used to help predict the risk of neurodevelopmental impairment.

CMR to Predict Patients with Biventricular Repair in Borderline Single Ventricle2
The study utilized multimodality imaging to guide surgical planning and predict surgical outcomes. Of 67 infants with borderline small left ventricles (LV), 54 achieved biventricular repair (BVR). Of note, 83% of the BVR patients had the historic LV volume cut-off of <20ml/m2 by echocardiography. Compared to CMR, echocardiography appears to underestimate the volume by approximately 17ml/m2. Ascending aorta (AAo) flow was significantly higher in the BVR group. Factors that were considered to determine BVR candidacy included the presence of endocardial fibroelastosis, AAo flow and mitral valve z-score. The transplant free survival in BVR group was 77% at 10 years. Patients without coarctation and patients with very low AAo flow were at increased risk of mortality.

The Risk of COVID-19 Vaccine Related Myocarditis in Young People3,4
This study from pooled data of 23 million Nordic residents showed an increased incidence of myocarditis and pericarditis after the first and second dose of mRNA vaccine. The risk was highest among males 16-24 years of age after the second dose. Both the BNT162b2 (Pfizer) and mRNA-1273 (Moderna) vaccines may result in myo- and/or pericarditis (4-7 excess cases in 28 days per 100,000 patients and 9-28 cases in 28 days per 100,000 patients, respectively). Recovery is expected in the vast majority of patients, and most patients are cleared for exercise participation by 90 days post diagnosis.

Outcomes of TPVR5
The recent availability of the Harmony TPV (Medtronic, Inc.) and Alterra-Sapien S3 (Edwards Lifesciences LLC) systems for native right ventricular outflow tracts has once again placed TPVR center stage in the pediatric and congenital interventional cardiology world. This study followed 2,476 patients that underwent TPVR at 15 international centers, with median age at TPVR being 20.5 years. Median follow-up was 2.8 years and approximately 40% of the cohort was followed for >8 years. The estimated cumulative incidence of death at 8-years post TPVR was found to be 8.9%. Reintervention was required on 258 patients: 53% of them undergoing surgical replacement of the conduit/valve, 29% requiring a valve-in-valve procedure, and 19% undergoing balloon angioplasty. The estimated cumulative reintervention rate at 8 years was 25.1%. Not surprisingly, reintervention was more common in younger patients. It was also more common in patients with a Ross-procedure and those with a TPVR-gradient immediately post-implant. The authors concluded that the rates of survival and reintervention between TPVR and surgical conduit/valve replacement were comparable. This study provided much needed longitudinal information about outcomes for what has become a flagship procedure in pediatric and congenital interventional cardiology.

Indeed, 2022 was an exciting year for the field of pediatric cardiology and adult CHD with many innovative and excellent research studies.


  1. Sadhwani A, Wypij D, Rofeberg V, et al. Fetal brain volume predicts neurodevelopment in congenital heart disease. Circulation 2022;145:1108-19.
  2. Kang SL, Chaturvedi RR, Wan A, et al. Biventricular repair in borderline left hearts: insights from cardiac magnetic resonance imaging. JACC Adv 2022;1:1–11.
  3. Kracalik I, Oster ME, Broder KR, et al. Outcomes at least 90 days since onset of myocarditis after mRNA COVID-19 vaccination in adolescents and young adults in the USA: a follow-up surveillance study. Lancet Child Adolesc Health 2022;6:788-98.
  4. Karlstad Ø, Hovi P, Husby A, et al. SARS-CoV-2 vaccination and myocarditis in a Nordic cohort study of 23 million residents. JAMA Cardiol 2022;7:600-12.
  5. McElhinney DB, Zhang Y, Levi DS, et al. Reintervention and survival after transcatheter pulmonary valve replacement. J Am Coll Cardiol 2022;79:18-32.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, COVID-19 Hub, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement, Echocardiography/Ultrasound, Magnetic Resonance Imaging

Keywords: Heart Defects, Congenital, COVID-19, COVID-19 Vaccines, Pulmonary Valve, Magnetic Resonance Spectroscopy, Pregnancy, Myocarditis, Mitral Valve, Heart Ventricles, Magnetic Resonance Imaging, Echocardiography, Social Class, Treatment Outcome

< Back to Listings