Contemporary Approaches to Congenital Heart Disease: Pearls Gleaned from ACC20/WCC Virtual

These "10 points to remember" are a compilation of key items discussed in the Congenital Heart Disease learning pathway presentations during ACC20/WCC Virtual. We hope you find this summary valuable in evaluating and managing patients with congenital heart disease.

  1. Obesity is multifactorial, but a decline in physical activity as children grow is a major etiologic contributor. About 60% of adult patients with Congenital Heart Disease (CHD) are overweight or obese, especially in African-American and Hispanic populations. In conjunction with a sedentary lifestyle, obesity increases the incidence of coronary atherosclerosis and premature exogenous death in this population.1
  2. Exercise is still the best medicine. In most cases, exercise prescription rather than restriction is preferred, barring hemodynamically significant residual lesions. Physical activity improves overall health, including cardiac function and lung capacity. It also increases self-confidence, independence and psycho-social well-being. Well visits for children with CHD should include BMI assessment, as well as counseling on diet and exercise.1,2
  3. Exercise has been found to be particularly beneficial even in single ventricle patients palliated with the Fontan procedure, who tend to have myopenia. Skeletal muscle contraction acts as a pump that contributes to stroke volume. By increasing skeletal muscle mass, especially to the lower extremities, resistance training improves cardiac output, exercise capacity, and tolerance to positive airway pressure in Fontan physiology. Respiratory muscle training improves ventilatory efficiency, peak exercise capacity, and stroke volume. Regular sports participation in this population is also associated with improved single ventricle systolic contractility and hepatic function. Obesity, and its commonly associated obstructive sleep apnea, increase the risk for Fontan failure.1,3, 4
  4. Three-dimensional printing is becoming increasingly important in creating a procedural "game plan," particularly in planning septation in complex ventricular septal defects (VSDs) in double outlet right ventricle, AV valve repairs, or optimization of Fontan hemodynamics. Virtual reality displays can overlay images from cross sectional modalities or echocardiography onto fluoroscopy displays.5,6,7
  5. Gut dysbiosis increases cardiovascular risk. In neonates with complex CHD, alterations in gut flora, possibly exacerbated by intestinal hypoperfusion, broad spectrum antibiotic administration, indwelling feeding tubes, cardio-pulmonary bypass and gut inflammation, may increase the risk for necrotizing enterocolitis (NEC). Probiotic and inulin administration may reduce the incidence of nosocomial sepsis, NEC and death in cyanotic CHD.8
  6. Many Fontan patients are entering their fourth or fifth decades of life. Excluding pre-Fontan attrition and operative mortality, the 30-year survival of this population is about 48%. As they age, patients palliated with the Fontan for complex single ventricle disease have a high morbidity. Only about 40% are free of adverse events at age 40. These include protein losing enteropathy, plastic bronchitis, thrombo-embolism, cirrhosis and the need for pacemaker or re-intervention. Many develop chylous effusions from impaired lymphatic re-absorption, progressive cyanosis due to the development of veno-venous collaterals, recurrent cerebrovascular accidents resulting in declining cognitive and executive function and neurohormonal derangements that may contribute to late cardiac dysfunction, and chronically low cardiac output.7,9
  7. Negative pressure ventilation in biphasic mode, currently used for acute respiratory distress syndrome (ARDS), chronic obstructive pulmonary disease (COPD), neuromuscular disorders, and cystic fibrosis, is touted as promising novel therapy for Fontan patients. It may increase pulmonary blood flow and consequently, cardiac output by decreasing mean intrathoracic pressure as measured by magnetic resonance imaging (MRI). In biphasic mode, negative pressure during inspiration maximizes chest wall expansion by creating adequate suctional driving forces. Positive pressure during expiration actively pushes the chest wall and abdomen, providing effective chest recoil and facilitating gas exchange. These ventilators are well tolerated in a small cohort, allowing patients to ambulate, talk and eat while fully supported, with minimal baro-trauma.9
  8. Two thirds of Fontan patients with common AV valves and about half with systemic tricuspid valves develop significant systemic AV valve regurgitation by age 25, increasing the risk of early death or transplantation by a factor of 2.4. Achieving a durable long-term result with valve repair is difficult and valve replacement at the subsequent operation should be considered. The optimal timing for repair and the proper valvuloplasty techniques are still topics of ongoing debate.10
  9. Biventricular hearts, deemed "non-septatable" and thus palliated as Fontan single ventricles, do not fare better than single ventricle Fontans (i.e., "No added value of a second ventricle in the Fontan circulation"). It is felt that the left ventricle (LV), even if moderately hypoplastic, can be harnessed as the sub-pulmonary ventricle when it cannot be committed to the aorta. Patients with heterotaxy and complex systemic and pulmonary venous drainage may also be deemed "non-septatable" despite two good sized ventricles. Some of these patients would benefit for consideration for biventricular conversion or even "1 ½ V repairs." This is felt to be preferable to Fontan physiology long term, theoretically extrapolating data from patients with late congenitally corrected transposition of the great arteries (CCTGA), in whom outcomes, though not ideal, are better than Fontan physiology. Three-dimensional heart models are useful to assess feasibility of Fontan conversion.7
  10. In Fontan patients, there is evidence that thromboprophylaxis is beneficial to prevent thromboembolism (cerebrovascular, intracardiac, pulmonary, systemic or intracoronary), which has an incidence of up to about 40%. However, some patients still develop thromboembolism despite prophylaxis. There is no evidence that warfarin or aspirin is superior to the other. Novel oral anticoagulants (NOACs) are promising but require more long-term data on safety and efficacy.11

References

  1. Impact of Obesity in Children with CHD. Presented by Dr. Meryl Cohen at the American College of Cardiology/World Congress of Cardiology Virtual Annual Scientific Session (ACC20/WCC), March 28, 2020 (Slides On Demand).
  2. Psychological Impact of Exercise Restrictions. Presented by Dr. Nicole Dempster at the American College of Cardiology/World Congress of Cardiology Virtual Annual Scientific Session (ACC20/WCC), March 28, 2020 (Slides On Demand).
  3. Exercise in the Fontan Patient: the Other Medication. Presented by Dr. Rachel Cordina at the American College of Cardiology/World Congress of Cardiology Virtual Annual Scientific Session (ACC20/WCC), March 28, 2020 (Slides On Demand).
  4. Exercise and Fontan: My Experience. Presented by Ms. Alicia Wilmoth at the American College of Cardiology/World Congress of Cardiology Virtual Annual Scientific Session (ACC20/WCC), March 28, 2020 (Slides On Demand).
  5. Mixed Reality And 3D Printing: Gimmick or Groundwork for Innovation. Presented by Dr. Laura Olivieri at the American College of Cardiology/World Congress of Cardiology Virtual Annual Scientific Session (ACC20/WCC), March 28, 2020 (Slides On Demand).
  6. State of the Art Imaging Evaluation for Single Ventricle Function. Presented by Dr. Cesar J. Herrera at the American College of Cardiology/World Congress of Cardiology Virtual Annual Scientific Session (ACC20/WCC), March 28, 2020 (Slides On Demand).
  7. From Single V to Two V: Using the Sub-pulmonary LV. Presented by Dr. Hani K. Najm at the American College of Cardiology/World Congress of Cardiology Virtual Annual Scientific Session (ACC20/WCC), March 28, 2020 (Slides On Demand).
  8. What Does the Gut Microbiome Have to Do with the Heart? Innovations to Personalize the Care of Congenital Heart Disease. Presented by Dr. W.H. Wilson Tang at the American College of Cardiology/World Congress of Cardiology Virtual Annual Scientific Session (ACC20/WCC), March 28, 2020 (Slides On Demand).
  9. Negative Pressure Ventilation as Novel Therapy to Treat Fontan Complications. Presented by Dr. Pradeepkumar Charla at the American College of Cardiology/World Congress of Cardiology Virtual Annual Scientific Session (ACC20/WCC), March 28, 2020 (Slides On Demand).
  10. The Impact of AV Valve Failure in the Fontan Circulation. Presented by Dr. Yves d'Udekem at the American College of Cardiology/World Congress of Cardiology Virtual Annual Scientific Session (ACC20/WCC), March 28, 2020 (Slides On Demand).
  11. Aspirin, Warfarin, NOAC's: What Should We Use for the Fontan? Presented by Dr. Rukhmi Bhat at the American College of Cardiology/World Congress of Cardiology Virtual Annual Scientific Session (ACC20/WCC), March 28, 2020 (Slides On Demand).

Clinical Topics: Anticoagulation Management, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Diabetes and Cardiometabolic Disease, Invasive Cardiovascular Angiography and Intervention, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Interventions, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement, Interventions and Structural Heart Disease

Keywords: ACC Annual Scientific Session, acc20, Heart Defects, Congenital, Fontan Procedure, Warfarin, Double Outlet Right Ventricle, Inulin, Heart Ventricles, Respiratory Distress Syndrome, Adult, Anticoagulants, Stroke Volume, Dysbiosis, Tricuspid Valve, Heart Septal Defects, Ventricular, Protein-Losing Enteropathies, Sedentary Lifestyle, Cross-Sectional Studies


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