The complex patient vad transplant exchange or hospice
- 1. The Complex Patient: VAD, Transplant, Exchange or Hospice? Joseph G. Rogers, MD Professor of Medicine Division of Cardiology Duke University American College of Cardiology Annual Scientific Sessions March 14, 2015 San Diego, CA Disclosures: None Lee Goldberg, MD Professor of Medicine Division of Cardiology University of Pennsylvania
- 2. History of Present Illness 76 y/o man presents to ED for evaluation of recurrent cough syncope • Multiple episodes over previous several months • Several months of progressive heart failure symptoms • Orthopnea/PND • Abdominal distention • PMH • LV dysfunction • Prostate Cancer: Radical Prostatectomy 1998 • HTN • Gout • CKD Stage 3: Cr 1.8, GFR 45 • Paroxysmal atrial fibrillation • LE neuropathy attributed to prior back surgery
- 3. Meds/Exam • Carvedilol 3.125 mg bid, allopurinol 200 mg daily, temisartan 80 mg daily, gabapentin 600 mg at bedtime, warfarin, atorvastatin 10 mg qHS, furosemide 40 mg daily • No tobacco, social ETOH, retired executive, widower in relationship • Fx: CAD, RAD, alzheimer’s • Afeb-84-131/94 – WD/WN, NAD. Appeared younger than stated age – Clear lungs – Irregular rhythm, no murmur or gallop, JVP 4 cm above clavicle at 450 – Bilateral LE edema
- 4. Laboratory Evaluation • Na=136, K=3.8, BUN/Cr= 38/1.8. • INR=2.9, Hct=42% • CK=339 with MB=10. Troponin T=0.10 • NT-proBNP=7031 • TSH=3.7 • UA: no proteinuria • Blood type: AB
- 5. ECG
- 6. Echo
- 7. Cardiac Catheterization (after diuresis) • Normal coronary arteries • Hemodynamics – RA=5, PA=35/13, PCWP=14 – CI=1.7 – SVO2=55%
- 8. Cardiac Biopsy • Negative SPEP/UPEP • Negative bone marrow • No gene analysis performed
- 9. Cardiac Amyloid • Three types – AL – associated with plasma cell dyscrasia (multiple myeloma) – TTR – mutant transthyretin protein – Familial • Multiple mutations leading to variable phenotypes • Val122Ile is associated with predominantly cardiac involvement – 3 to 4% of African Americans – TTR – wildtype – Senile amyloid – usually cardiac only
- 10. Genetics Determine Phenotype Heart Fail Rev (2015) 20:163–178
- 11. Clinical Syndrome • Heart failure with preserved ejection fraction • Heart failure with decreased ejection fraction – Intolerance to vasodilators – Intolerance to beta blockers • Arrhythmias • Peripheral neuropathy • Orthostatic hypotension • AL amyloid can impact many organs • TTR depending on genotype can impact kidney and other tissues
- 12. Prognosis • Onset of advanced heart failure symptoms – 6 month survival in AL amyloid – 43 months in wild-type TTR – 24 months in familial TTR • AL amyloid – Chemotherapy to suppress light chains can lead to some regression • TTR amyloid – No therapy to slow disease • Drugs now in clinical trial
- 13. Prognosis Heart Fail Rev (2015) 20:163–178
- 14. Treatment • AL Amyloid – Treat the underlying plasma cell dyscrasia – Heart transplant +/- stem cell transplant • TTR – Organ transplantation - heart/liver for familial – Transthyretin stabilizers (diflunisal, tafamidis, AG-1) – TTR silencers (ALNATTR02, ISIS-TTR(Rx)) – Degraders of amyloid fibrils (doxycycline/TUDCA)
- 15. Treatment Targets - TTR Heart Fail Rev (2015) 20:163–178
- 16. Hospital Course • Develops more NSVT • Develops worsening dyspnea, hypotension and renal insufficiency despite volume • Started on dopamine
- 17. Discussion Points • Should this man be enrolled in a clinical trial for the treatment of TTR amyloid? • Should this man be enrolled in hospice? • Should he receive a DT LVAD? • Should he receive an extended criteria transplant? – Age – Amyloid – Renal insufficiency
- 20. Case Conclusion • Transplanted < 1 month after listing • Hospital course – Extubated day of transplant – To floor POD 1 – Discharged from hospital POD 5 • 2 readmissions – 24 hours for hydration – Laminectomy • Just celebrated 3 year anniversary – Rows daily – Cr 1.8 – Normal coronary arteries