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Pediatric and Congenital Heart Disease: Compassionate Allowances

This testimony discusses the goals of the Compassionate Allowances program for severe forms of cardiovascular disease in children, including diagnosis, treatment, functional limitations, and emerging science. It also highlights the need for fast-tracking applications, reducing administrative costs, and developing a list of diagnoses to better discriminate children meeting disability criteria.

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Pediatric and Congenital Heart Disease: Compassionate Allowances

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  1. Pediatric and Congenital Heart Disease:Compassionate Allowances Geoffrey L. Rosenthal, MD, PhD November 9, 2010

  2. Disclosures • Financial conflicts of interest: None

  3. Compassionate Allowances :Goals of Testimony • Identify most severe forms of cardiovascular disease in children • Discuss diagnosis and treatment of these disorders • Provide insight into the pervasiveness of “medicine” in the lives of families • Discuss functional limitations/expectations for children with these diagnoses • Address emerging science and research

  4. Compassionate Allowances:Program Goals • Reduce backlog of cases by fast tracking applications • Reduce administrative costs of processing claims • Develop “List” of diagnoses which better discriminate children meeting cardiovascular disability criteria • Maintain a low rate of denying benefits when they should be allowed, and of allowing benefits when they should be denied

  5. Compassionate Allowances:Program Goals • Identify diseases that are certain or near certain to cause disability or death within 12 months • Identify diseases that, by definition, cause disability

  6. Compassionate Allowances:SSA Suggested Dx for Discussion • Single ventricle • Hypoplastic Left Heart Syndrome (HLHS) • Aortic valve atresia • Tricuspid valve atresia • Pulmonary atresia with intact ventricular septum (PAIS or PAIVS) • Long QTc Syndrome with aborted sudden death • Childhood myocardial infarction • Heart transplantation

  7. Compassionate Allowances:Finding a Framework for “Severe” • “Palliated” Cardiovascular Malformations, and “Repaired” Malformations with lifelong sequelae • Single ventricle and complex two ventricle lesions • Channelopathies • Cardiomyopathies • Congenital Heart Disease with comorbidities • Congenital Heart Disease requiring specific treatments/events

  8. Palliated and Repaired CHD, <12 Month Old, Single Ventricle • Hypoplastic left heart syndrome (HLHS) • Aortic atresia, Mitral atresia, AA/MA • Pulmonary atresia with intact ventricular septum • Tricuspid atresia (all subtypes) • Unbalanced atrioventricular canal (all subtypes) • Double inlet left ventricle • Some forms of double outlet right ventricle • Single ventricle with indeterminate morphology

  9. All Forms of Single Ventricle • Diagnostic determination of single ventricle can be made with a very high degree of certainty using echocardiography • Resting cyanosis (paO2 < 60 Torr) pre-op and post-op while infants • Clinically significant risk of death in the first year of life (approximately 10-50+%) • Usually require 3 or more hospitalizations in first year of life for diagnosis, stabilization, surgery, and/or cardiac catheterization

  10. Palliated and Repaired CHD, <12 Month Old, Complex Two Ventricles • Tetralogy of Fallot • With pulm. atresia, absent pulm. valve syndrome, discontinuous or hypoplasticpulm. arteries • Transposition of the great arteries (d-TGA and l-TGA, with or without other cardiac lesions) • Pulmonary atresia with ventricular septal defect and multiple aortopulmonary collaterals • Remaining forms of double outlet right ventricle • Critical aortic valve stenosis • Shone’s complex • Critical pulmonary valve stenosis

  11. Palliated and Repaired CHD, <12 Month Old, Complex Two Ventricles • Total anomalous pulmonary venous return (all types) • Interrupted aortic arch (all types) • Truncus arteriosus (all types) • Ebstein’s anomaly diagnosed in infancy (with or without associated lesions) • Heterotaxy syndrome (all types) • Pulmonary vein stenosis/sclerosis involving 2 or more pulmonary veins

  12. Complex Two Ventricles, Functional Sequelae Common • Diagnosis of complex two ventricle lesions can be made with a very high degree of certainty using echocardiography • Precise anatomical diagnosis may require cMRI or cardiac catheterization • Resting cyanosis (paO2 < 60 Torr) pre-op for most, low cardiac output for remaining

  13. Complex Two Ventricles, Functional Sequelae Common • Clinically significant risk of death in the first year of life (approximately 5-50+%) • Usually require 2 or more hospitalizations in first year of life for diagnosis, stabilization, surgery, and/or cardiac catheterization • Hospitalizations are often prolonged (LOS > 2 weeks) • Sequelae, residual lesions, need for nutritional support, need for home care is common

  14. Cardiovascular Genetics

  15. Inherited Channelopathies • Long QT syndromes • Brugada syndrome variants • Atrial arrhythmia syndromes • Short QT syndrome • Catecholaminergic ventricular tachycardia

  16. Inherited Channelopathies • Diagnosis very reliable when based upon clinical features, family history, ECG, heart rhythm assessment, and genetic testing • Clinically significant risk of arrhythmia, syncope, and death • Most require medications and lifestyle modifications • Many have associated developmental/functional disabilities • Most require electrophysiology procedures, pacemaker, and/or internal cardioverter-defibrillator

  17. Inherited Channelopathies • Devices require lifestyle modification to prevent lead fracture and device malfunction • Devices may be associated with psychological symptoms (body image, anxiety, depression) • Often limit age-appropriate abilities at home, in school, and in the community • Hospitalizations may be prolonged (LOS > 2 weeks)

  18. Cardiomyopathies • Autosomal, Gonosomal, or Mitochondrial • Acquired (infectious, post-infarction, post-bypass, toxic, nutritional) • Dilated • Hypertrophic • Restrictive • Arrhythmogenic right ventricular dysplasia • Many occur within defined systemic syndromes

  19. Childhood Myocardial Infarction • Anomalous left coronary arising from the pulmonary artery (ALCAPA) • Kawasaki Disease with coronary artery aneurysms • Anomalous left coronary arising from the right cusp and passing between the aorta and the pulmonary artery • The causes of myocardial infarction in children are different than in adults, but the outcomes are similar

  20. Cardiomyopathies • Diagnosis very reliable when based upon clinical features, physiological assessment, family history, echocardiography, and genetic testing • Significant risk of arrhythmia, syncope, and sudden death • Most require medications, lifestyle modifications • Most have significant functional limitations • Many have associated developmental disabilities • Some require palliative surgery, internal cardioverter-defibrillator

  21. Cardiomyopathies - Transplantation • Pediatric Heart Transplantation – Medical Urgency Status Codes • Status 1A and 1B patients meet criteria for compassionate allowance prior to transplant

  22. Cardiomyopathies - Transplantation • Status 1A - “Registrant less than 18 yrs of age and meets at least one of the following criteria: (a) requires assistance with a ventilator; (b) requires assistance with a mechanical assist device; (c) requires assistance with a balloon pump; (d) is less than 6 months old with congenital or acquired heart disease exhibiting reactive pulmonary hypertension at greater than 50% of systemic level; (e) requires infusion of high dose or multiple inotropes; or (f) meets none of the criteria specified above but has a life expectancy without a heart transplant of less than 14 days”

  23. Extracorporeal Membrane Oxygenator (ECMO) - Bypass

  24. Cardiomyopathies - Transplantation • Status 1B - “Registrant who (a) requires infusion of low dose single inotropes, (b) is less than 6 months old and does not meet the criteria for Status 1A, or (c) exhibits growth failure (see OPTN policies for definition).

  25. Congenital Heart Disease with Co-Morbidities • Prematurity <37 weeks gestation • Neuroradiographic signs of injury • Microcephaly • Post-operative seizures • Developmental delay identified before 1 year of age • Multiple congenital anomalies • Syndromes associated with developmental delay and functional impairment (Down, Williams, DiGeorge, CHARGE, Noonan, Jacobsen)

  26. Syndromes and Cardiovascular Anomalies

  27. Congenital Heart Disease requiring Specific Treatments/Events • Length of stay in ICU > 2 weeks • Need for Cardiopulmonary resuscitation • Need for mechanical circulatory support (ECMO, VAD) • Need for tracheostomy • Need for continuous infusion of pulmonary vasodilators or inotropes • Prior fetal intervention

  28. Thank you, Questions?

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