Ileana Piña, MD, MPH American Heart Association

1. Compassionate Allowances Outreach HearingCardiovascular Disease & Multiple Organ TransplantsSocial Security AdministrationNovember 9, 2010 Ileana Piña, MD, MPH American Heart Association

2. Cardiovascular Disease • 81 million American adults – more than 1 in 3 – have one or more types of CVD • # 1 killer of Americans – including women • Includes high blood pressure, coronary heart disease, heart failure, stroke, and congenital cardiovascular defects

3. Social Security Disability • CVD can affect ability to work, comply with medical care, engage in activities of daily living • Complex process; application denied or require multiple applications • 24-month waiting period for Medicare • CVD events may occur before eligibility (hospitalizations or death)

4. Heart Failure • Often the final pathway of a multitude of heart disorders • Affects 5.8 million Americans • Chronic and progressive • Affects children and adults • One of the worst sequelae of any heart disease • Many hospitalizations and high mortality • Cannot be cured

5. HF: Highest 30-day Rehospitalization Rate Jencks et al. N Engl J Med 2009;360:1418-28.

6. Outcomes in Patients Hospitalized with HF Mortality Hospital Readmissions 100 100 75 75 50% 50% 50 50 33% 20% 25 12% 25 0 0 6 Months 30 Days 30 Days 12 Months 5 Years Mean LOS: 6.5 days Annual mortality rate- NYHA class III HF- 12% [COPERNICUS DATA] NYHA class II HF- 7% [SCD-HeFT DATA] Fonarow, GC. Rev Cardiovasc Med. 2002;3(suppl 4):S3 Jong P et al. Arch Intern Med. 2002;162:1689

7. Causes of Hospital Readmission for CHF Over 2/3 of HF Hospitalizations Preventable Diet Noncompliance 24% Rx Noncompliance 24% 16% Inappropriate Rx 17% Other 19% Failure to Seek Care Post d/c monitoring Annals of Internal Medicine 122:415-21, 1995

8. Heart Failure Symptoms • Shortness of breath • Swelling in feet, ankles, legs, or abdomen • Weight gain • Tiredness, fatigue • Persistent coughing or wheezing • Lack of appetite or nausea • Confused or impaired thinking • Symptoms may be present even at rest

9. Evaluating Heart Failure Status • LV Function • Non-invasive imaging (MRI, Transthoracic Echo, Doppler) • Cardiac catheterization • BNP and NT-Pro-BNP • Predictive of mortality; CV events; overall prognosis • Highly variable; lacks specificity; false positives • Functional Capacity • Most accurate is cardiopulmonary exercise testing • 6 minute walk to determine function of sicker patients • Prognosis for Improvement • No reliable scales or measures

10. HF: Compassionate Allowances • With medical therapy: • 6-min walk distance less than 309 feet • VO2 max less than 50% of predicted • Or are dependent upon inotrope therapy or mechanical support  • Cardiac rehab may not available or not covered • Extremely high risk of mortality • Should be considered for Compassionate Allowances List

11. Congenital Cardiovascular Disease • 9 out of every 1,000 births • Severe malformations – complete absence of one or more chambers (hypoplastic left heart syndrome, single ventricle) or valves (aortic, tricuspid or pulmonary atresia) • Increases the risk of pulmonary hypertension, arrhythmias, infective endocarditis, congestive heart failure, and the need for anticoagulants

12. Impact of Congenital Heart Disease • Severe functional limitations • Highly specialized care required; frequent office visits and hospitalization; complicated medicine regimens • Often full-time care giver support • Developmental delays or learning difficulties • Adults may not be able to work

13. Symptomatic Progression for Adults with Congenital Heart Disease • Variable • Worst Prognosis: single ventricle or systemic right ventricle • Frequently develop HF as adults

14. Heart Transplantation • Waiting for a heart transplant: • Have advanced heart failure and a poor prognosis • Those most likely to be offered a heart have a 50% 1-year mortality • Most are unable to drive and require transportation; usually cannot work • May have constant infusion of an intravenous drug

15. Heart Transplantation • Waiting for a heart transplant: • 20 - 25% of children do not receive an organ in time due to severity of illness and donor scarcity • Reasons for transplants in children are age-dependent: • Birth – 1 year: congenital • 1 – 10 years: congenital and cardiomyopathy

16. Heart Transplantation Issues • VAD – bridge to transplant – wound care and battery change assistance • Rejection, immunosuppression, co-morbidities and malignancies • Impaired exercise capacity • Cardiac rehab may not available • Medication side effects (muscle weakness; drug induced diabetes, obesity, and high blood pressure)

17. The Disability Process • Severe CVD can result in: • Major life changes: physical, financial, professional, social, emotional, and psychological • Mobility and stamina challenges • Inability to work; career-ending; lack of income • Increasing dependence on others for care

18. The Disability Process • Hurdles applying for disability benefits: • Confusing and lengthy process • Denials and reapplications common • 2-year wait until eligible for Medicare • Rehabilitation not readily available • Patients are very ill, helpless on many levels • Timely assistance is critical

19. AHA Recommendations • Reduce the time between submission of disability application and approval of benefits • Offer temporary disability benefits for those with severe CVD until a complete review is completed • Reduce the 24-month Medicare waiting period

20. AHA Recommendations:Compassionate Allowances List • Advanced HF patients who are candidates for LVAD or heart transplant • Adults with CVD and class IV symptoms and cyanotic adult congenital heart disease where transplant is the only option • Class IV symptoms with intractable primary pulmonary hypertension where lung transplantation is the only hope

21. AHA Recommendations:Compassionate Allowances List • Class IV disease where a treatment option exists, (hypertrophic cardiomyopathy [surgery], sarcoid heart disease, amyloidosis, [? immunosuppression], prolonged QT interval with SCD [implanted defibrillator], etc.) • HF patients with a 6-min walk distance less than 309 feet, VO2 max less than 50% of predicted, or are dependent upon inotrope therapy or mechanical support 

22. AHA Recommendations:Compassionate Allowances List • Fulminant giant cell myocarditis in which urgent intervention is required • Lymphomatous invasion of the heart • Congenital heart disease with Eisenmenger physiology and pulmonary vascular disease • Congenital heart disease and NYHA Class 4 symptoms

23. AHA Recommendations:Compassionate Allowances List • Congenital heart disease and severe cyanosis (tetralogy of Fallot, transposition of the great arteries, tricuspid atresia, pulmonary atresia, truncus arteriosus and total anomalous pulmonary venous connection) • Congenital heart disease after Fontan palliative surgery and protein losing enteropathy • Congenital heart disease after Fontan with systolic or diastolic dysfunction

24. AHA Recommendations:Compassionate Allowances List • Transposition of the great vessels after Mustard procedure and RV dysfunction • Hypoplastic left heart syndrome • Valvular atresia – aortic, tricuspid, pulmonary valve • Single ventricle patients with congestive heart failure • Childhood myocardial infarction

25. Thank You Questions? American Heart Association www.heart.org