These "10 Points to Remember" are a compilation of key items discussed in the Pediatric and Congenital Heart Disease learning pathway presentations during ACC.21 Virtual. We hope you find this summary valuable in evaluating and managing patients with congenital heart disease.

You can still explore all the education from ACC.21 Virtual until December 31, 2021 with On Demand, offering access to practice-changing research and cutting-edge education with more than 250 sessions and 3,300 eAbstracts.

1. In post-op cardiac patients, whether to administer colloid or crystalloid, at what volume and at which rate, is still controversial. Protocols are based on regional or institutional practices, and still not evidence based. Within a physiologic range, fluid administration can vary without adverse consequences.¹
2. The early use of mechanical cardiac support (MCS) in acute decompensated dilated cardiomyopathy is controversial. Depending on resources, medical management alone may still be the first line of stabilization, reserving MCS for those in whom medical management fails. However, in those with progressive decline (INTERMACS Profile 2) a period of ventricular assist device (VAD) support prior to heart transplantation may mitigate end organ damage and improve waitlist survival and post-transplant outcomes, with declining stroke risk in the current era.^2,3
3. The goals of post-op sedation after cardiac surgery include pain relief and stabilization of vital signs with reduction of metabolic demands and care provider distress. Practices have changed over time, but currently include scheduled intravenous (IV) acetaminophen, continuous dexmedetomidine and continuous fentanyl with boluses as needed.⁴
4. In neonates with hypoplastic left heart syndrome (HLHS), except in cases associated with delayed diagnosis and cardiogenic shock, feeding is recommended in the pre-operative period and started cautiously as soon as feasible in the post-operative period. Human milk and a standardized algorithm for feeding advancement are recommended.⁵
5. In pregnancies associated with congenital heart disease (CHD), pre-conception stratification is useful to formulate a personalized risk assessment that is multi-disciplinary, prospective, and patient centered. This includes a thorough history and physical examination (H&P), electrocardiogram (ECG) and echocardiogram, with more testing recommended in patients in the WHO Class III category or who have high CARPEG II or ZAHARA scores, or in those who are physically inactive, with a poor functional status, who are desaturated with exercise or who have "high-risk" lesions. Impaired peak V02, chronotropic, blood pressure (BP) or desaturation response to exercise are associated with poor outcomes. Cross-sectional imaging is useful in Fontan, systemic right ventricles (RVs), severe valve regurgitation, coronary abnormalities, or aortopathies. Arrhythmias are common. Women with systemic RVs have high risk pregnancies and may develop irreversible decline in functional status, worsening tricuspid regurgitation, and RV dysfunction, with no reliable predictors of poor outcomes. Congestive heart failure (CHF) and arrhythmias may develop post-partum. A multi-disciplinary team is essential for delivery planning and comprehensive maternal-fetal assessment to identify placental insufficiency and other complications that may require early delivery.^6-9
6. Multisystem Inflammatory Syndrome in Children (MIS-C) is defined as fever, lab evidence of inflammation, multi-system involvement requiring hospitalization, with no alternative diagnosis and evidence of SARS COV2 infection or exposure within 4 weeks. It must be considered in any pediatric death associated with SARS COV2 infection. MIS-C is rare, with incidence that lags being COVID-19 prevalence. Cases with neutrophil-lymphocyte ratio >5, platelets <150k, and elevated c-reactive protein (CRP) are more likely to be MIS-C rather than isolated COVID-19. Being a hyperinflammatory disease, MIS-C will benefit from immunomodulators but "not everyone needs them". Genetic studies to inform predisposition and precision therapy for different phenotypes will be the wave of the future.¹⁰
7. In over 800 adult patients after repair of coarctation of the aorta (CoA) in childhood, there is accelerated attrition after the third decade. Hypertension was present in 60% and despite medications, only 18% had good control. Freedom from arch reintervention or reoperation was 71% and 88% respectively at 30 years and 47 and 84% at age 60. Risk factors include left ventricular outflow tract (LVOT) obstruction at initial repair, <1 year of age at repair, initial transcatheter intervention, and initial surgical patch repair. Freedom from aortic valve and ascending aorta intervention was 53% and 81% by age 70, risk factors for which are male gender and bicuspid aortic valves. Lifelong surveillance for these patients is recommended.¹¹ After repair of interrupted aortic arch in adults, morbidity is associated with psychosocial issues although "disability paradox" is not uncommon.¹²
8. After the arterial switch operation in dextro-transposition of the great arteries (d-TGA), the incidence for re-operation for the neo-aortic valve (native pulmonary) is low, with only a minority exhibiting progression of aortic insufficiency (AI). The degree of AI at the time of neonatal discharge correlates with late AI. Those with at least mild AI at the time of neonatal discharge have a three-fold higher risk of developing at least moderate AI after 15 years.¹³
9. Late outcome of both repaired and unrepaired perimembranous ventricular septal defects (VSDs) is not entirely benign. In the Belgian registry of over 250 adults, the incidence of endocarditis was 2.3% in unrepaired patients, sub-aortic stenosis occurred in 14% of repaired patients, at least moderate AI was seen in 5%, and a few patients (both repaired and unrepaired) required pacemaker placement. Some VSDs are associated with cardiomyopathy. Patients with VSDs require continued follow-up as they age.¹⁴
10. Eliminating the second re-screen for neonates who initially fail pulse oximetry screening for critical CHD maintains >99% specificity with only a .01% increase in false positives. Population studies are needed to enhance the algorithm.¹⁵

Hepatic fibrosis is universally present in Fontan after the first decade. Bridging fibrosis and thrombocytopenia are associated with worse outcomes.¹⁶

The transcatheter GORE® CARDIOFORM ASD Occluder device demonstrates favorable composite clinical success and a low complication rate. Its flexibility makes it more "heart friendly".¹⁷

In the current era, more interstage HLHS patients are discharged home on digoxin, with more high-risk patients as indicated by the NEONATE risk score. Digoxin use is associated with decreased interstage mortality and increased freedom from unplanned admission. More studies are needed to determine if there are specific sub-groups who will benefit from digoxin and the mechanism behind its effects.¹⁸

References

1. Controversies in the ICU Management of Congenital Heart Disease: Debate: Fluid IS NOT a drug. Presented by Dr. Anthony Rossi at the American College of Cardiology Virtual Annual Scientific Session (ACC.21), May 15, 2021 (Slides On Demand).
2. Controversies in the ICU Management of Congenital Heart Disease: Mechanical Assist Transplant. Presented by Dr. Justin Godown at the American College of Cardiology Virtual Annual Scientific Session (ACC.21), May 15, 2021 (Slides On Demand).
3. Controversies in the ICU Management of Congenital Heart Disease: Fluids, Pressors. Presented by Dr. Santiago Borasino at the American College of Cardiology Virtual Annual Scientific Session (ACC.21), May 15, 2021 (Slides On Demand).
4. Controversies in the ICU Management of Congenital Heart Disease: Sedation Management. Presented by Dr. Laura Ortman at the American College of Cardiology Virtual Annual Scientific Session (ACC.21), May 15, 2021 (Slides On Demand).
5. Controversies in the ICU Management of Congenital Heart Disease: Nutrition. Presented by Dr. Chitra Ravishankar at the American College of Cardiology Virtual Annual Scientific Session (ACC.21), May 15, 2021 (Slides On Demand).
6. Nuts & Bolts of Pregnancy and Congenital Heart Disease: Are you Ready? -- Risk Stratification Prior to Conception. Presented by Dr. Abigail Khan at the American College of Cardiology Virtual Annual Scientific Session (ACC.21), May 15, 2021 (Slides On Demand).
7. Nuts & Bolts of Pregnancy and Congenital Heart Disease: RV There yet? -- Pregnancy and the Systemic RV. Presented by Dr. Sara Partington at the American College of Cardiology Virtual Annual Scientific Session (ACC.21), May 15, 2021 (Slides On Demand).
8. Nuts & Bolts of Pregnancy and Congenital Heart Disease: Cardiovert, Medicate or Ablate? -- Arrhythmias in Pregnancy. Presented by Dr. Anna Kamp at the American College of Cardiology Virtual Annual Scientific Session (ACC.21), May 15, 2021 (Slides On Demand).
9. Nuts & Bolts of Pregnancy and Congenital Heart Disease: What Cardiologists Should Know - the MFM Perspective. Presented by Dr. Diana Wolfe at the American College of Cardiology Virtual Annual Scientific Session (ACC.21), May 15, 2021 (Slides On Demand).
10. Management of Pediatric Cardiology and Cardiac Surgery in the Post-COVID World: Multisystem Inflammatory Syndrome in Children: Where Are We Now? Presented by Dr. George Ofori-Amanfo at the American College of Cardiology Virtual Annual Scientific Session (ACC.21), May 16, 2021 (Slides On Demand).
11. Repair Is Not Correction: Long Term Follow-up of Surgery for Congenital Heart Disease: Long-term Mortality and Cardiovascular Burden for Adult Survivors of Coarctation of the Aorta. Presented by Dr. Melissa Lee at the American College of Cardiology Virtual Annual Scientific Session (ACC.21), May 16, 2021 (Slides On Demand).
12. Repair Is Not Correction: Long Term Follow-up of Surgery for Congenital Heart Disease: Self-reported Functional Health Status Following Interrupted Aortic Arch Repair: A Congenital Heart Surgeons' Society Study. Presented by Dr. Anusha Jegatheeswaran at the American College of Cardiology Virtual Annual Scientific Session (ACC.21), May 16, 2021 (Slides On Demand).
13. Repair Is Not Correction: Long Term Follow-up of Surgery for Congenital Heart Disease: Long-term Fate of the Aortic Valve After an Arterial Switch Operation. Presented by Dr. Mauro Lo Rito at the American College of Cardiology Virtual Annual Scientific Session (ACC.21), May 16, 2021 (Slides On Demand).
14. Repair Is Not Correction: Long Term Follow-up of Surgery for Congenital Heart Disease: Long-Term Outcome of Patients With Perimembranous Ventricular Septal Defect: Results From the Belgian Registry on Adult Congenital Heart Disease. Presented by Dr. Werner Budts at the American College of Cardiology Virtual Annual Scientific Session (ACC.21), May 16, 2021 (Slides On Demand).
15. Highlighted Original Research: Pediatric and Congenital Heart Disease and the Year in Review: The Impact of Newborn Pulse Oximetry Screening at a Community Hospital: an Eight-year Experience. Presented by Dr. Bryanna Schwartz at the American College of Cardiology Virtual Annual Scientific Session (ACC.21), May 17, 2021 (Slides On Demand).
16. Highlighted Original Research: Pediatric and Congenital Heart Disease and the Year in Review: Clinical Features Associated with Bridging Fibrosis in Adolescent Patients with Fontan-associated Liver Disease. Presented by Dr. Neil Patel at the American College of Cardiology Virtual Annual Scientific Session (ACC.21), May 17, 2021 (Slides On Demand).
17. Highlighted Original Research: Pediatric and Congenital Heart Disease and the Year in Review: Outcomes of Secundum Atrial Septal Defect Closure with the New GORE Cardioform ASD Occluder – Results from the Continued Access GORE Assured Clinical Trial. Presented by Dr. Athar M. Qureshi at the American College of Cardiology Virtual Annual Scientific Session (ACC.21), May 17, 2021 (Slides On Demand).
18. Highlighted Original Research: Pediatric and Congenital Heart Disease and the Year in Review: The Impact of Digoxin Use on Interstage Mortality in the Current Era. Presented by Dr. Rachel Klausner at the American College of Cardiology Virtual Annual Scientific Session (ACC.21), May 17, 2021 (Slides On Demand).

Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, COVID-19 Hub, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Prevention, Valvular Heart Disease, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Aortic Surgery, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Cardiac Surgery and VHD, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Interventions, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement, Acute Heart Failure, Heart Failure and Cardiac Biomarkers, Heart Transplant, Mechanical Circulatory Support, Interventions and Structural Heart Disease, Hypertension

Keywords: ACC21, ACC Annual Scientific Session, Heart Defects, Congenital, Pediatrics, Pregnancy, Infant, Newborn, COVID-19, SARS-CoV-2, Patient Discharge, C-Reactive Protein, Acetaminophen, Dexmedetomidine, Aortic Valve, Aortic Coarctation, Heart-Assist Devices, Hypoplastic Left Heart Syndrome, Tricuspid Valve Insufficiency, Shock, Cardiogenic, Cardiomyopathy, Dilated, Pregnancy, High-Risk, Blood Pressure, Digoxin, Placental Insufficiency, Aorta, Thoracic, Blood Platelets, Heart Ventricles, Severe Acute Respiratory Syndrome, Heart Transplantation, Aortic Valve Stenosis, Heart Failure, Endocarditis, Heart Septal Defects, Ventricular, Arrhythmias, Cardiac, Electrocardiography, Risk Assessment, Hypertension, Risk Factors, Thrombocytopenia, Registries, Immunologic Factors, Inflammation, Liver Cirrhosis, Lymphocytes, Patient-Centered Care, Pacemaker, Artificial, Stroke, Coenzyme A, World Health Organization

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