36 th Annual Advances in Heart Disease Transthyretin Amyloid - - PDF document

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36 th Annual Advances in Heart Disease Transthyretin Amyloid - - PDF document

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12/7/19 36 th Annual Advances in Heart Disease Transthyretin Amyloid Cardiomyopathy: Screening and Management Strategies Mary O. Gray, M.D. Medical Director, Cardiology Clinical Services Zuckerberg San Francisco General 1 Disclosures No

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36th Annual Advances in Heart Disease Transthyretin Amyloid Cardiomyopathy: Screening and Management Strategies

Mary O. Gray, M.D. Medical Director, Cardiology Clinical Services

Zuckerberg San Francisco General

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Disclosures

No Financial Relationship With Any Commercial Interest

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Objectives

> Recognize clinical manifestations and natural history of cardiac transthyretin amyloidosis, a disabling and life-threatening disease. > Develop more effective screening and diagnostic strategies for transthyretin amyloid cardiomyopathy within everyday practice. > Implement standard therapy for heart failure with preserved EF and consider new treatments for this protein misfolding disorder.

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Heart Failure with Preserved LVEF: Evidence-Based Diagnosis

Circulation 2018;138:861-870

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Transthyretin: TTR TTR wild-type or mutant: TTRwt or TTRm Transthyretin Amyloidosis: ATTRwt or ATTRm Transthyretin Amyloid Cardiomyopathy: ATTR-CM

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Genotype & Phenotype: Mutant Transthyretin Amyloidosis

Akcea Therapeutics

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Screening for Transthyretin Amyloid Cardiomyopathy in Everyday Practice

J Am Coll Cardiol HF 2019;7(8):709-716

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Screening for Transthyretin Amyloid Cardiomyopathy

J Am Coll Cardiol HF 2019;7(8):709-716

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Screening for Transthyretin Amyloid Cardiomyopathy

J Am Coll Cardiol HF 2019;7(8):709-716

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Screening for Transthyretin Amyloid Cardiomyopathy

J Am Coll Cardiol HF 2019;7(8):709-716

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Transthyretin Cardiac Amyloidosis: AR Consortium

Circ Heart Fail 2019;12:e006075

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Transthyretin Cardiac Amyloidosis: AR Consortium

Circ Heart Fail 2019;12:e006075

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Transthyretin Cardiac Amyloidosis: AR Consortium

Circ Heart Fail 2019;12:e006075

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Circ Heart Fail 2019;12:e006075

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Ø 89 yo man with HTN. Referred due to shortness of breath. Ø Atrial fibrillation. Ventricular pacemaker implanted in 2005. Ø NM pharmacologic stress: No infarction. No inducible ischemia. Ø Apixaban 5, benazepril 20, furosemide 40, metoprolol 50.

Case Presentation

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Ø Chronic heart failure with preserved LV ejection fraction Ø ACC/AHA Stage C. NYHA Class III. Musculoskeletal conditions. Ø Normal volume status on loop diuretic and sodium restriction. Ø BP and HR management with medications and pacemaker.

Case Presentation

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Case Presentation

Echocardiogram

Normal LV cavity Mild concentric LVH LVEF 50 to 55% Dilated IVC collapses Severe tricuspid regurgitation. RA enlargement. Normal PA pressure.

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Case Presentation

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Case Presentation

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Case Presentation

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Ø IV administration of 13.9 mCi Tc-99m Pyrophosphate (PYP). Ø Planar imaging & SPECT-CT at 1-hour and 3-hour time points. Ø Semi-quantitative and quantitative analysis per ASNC 2016.

Case Presentation

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Case: Cardiac Sodium Pyrophosphate Scan

Anterior Chest 1 Hour Anterior Chest 3 Hours Visual Score 3

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Case: Cardiac Sodium Pyrophosphate Scan

Region of Interest @ 1 Hour Region of Interest @ 3 Hours

H/CL ratio 1.5

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Cardiac Sodium Pyrophosphate Scan

Fusion Coronal SPECT-CT Image

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Circ Heart Fail 2019;12:e006075

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ATTR-CM: Considerations for Heart Failure Therapy

> Loop diuretics are the mainstay of heart failure management. > Individuals may not tolerate beta blockers and ACE inhibitors. > Calcium channel blockers and digoxin are contraindicated. > Atrial fibrillation: Rate control, anticoagulation, rhythm control.

Ventricular Assist Device, Liver Transplant, Heart Transplant

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Transthyretin: TTR TTR wild-type or mutant: TTRwt or TTRm Transthyretin Amyloidosis: ATTRwt or ATTRm Transthyretin Amyloid Cardiomyopathy: ATTR-CM

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Transthyretin Amyloidosis Cardiomyopathy Trial (ATTR-ACT)

N Engl J Med 2018;379:1007-1016

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Transthyretin Amyloidosis Cardiomyopathy Trial (ATTR-ACT)

N Engl J Med 2018;379:1007-1016

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Transthyretin Amyloidosis Cardiomyopathy Trial (ATTR-ACT)

N Engl J Med 2018;379:1007-1016

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Transthyretin Amyloidosis Cardiomyopathy Trial (ATTR-ACT)

N Engl J Med 2018;379:1007-1016

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Transthyretin Amyloidosis Cardiomyopathy Trial (ATTR-ACT)

N Engl J Med 2018;379:1007-1016

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Transthyretin Amyloidosis Cardiomyopathy Trial (ATTR-ACT)

N Engl J Med 2018;379:1007-1016

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Transthyretin Amyloidosis Cardiomyopathy Trial (ATTR-ACT)

N Engl J Med 2018;379:1007-1016

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Objectives

> Recognize clinical manifestations and natural history of cardiac transthyretin amyloidosis, a disabling and life-threatening disease. > Develop more effective screening and diagnostic strategies for transthyretin amyloid cardiomyopathy within everyday practice. > Implement standard therapy for heart failure with preserved EF and consider new treatments for this protein misfolding disorder.

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