Infiltrative Heart Diseases - UCSF Medical Education Gray Amyloidosis.pdf · Infiltrative Heart...
Transcript of Infiltrative Heart Diseases - UCSF Medical Education Gray Amyloidosis.pdf · Infiltrative Heart...
12/19/15
1
Infiltrative Heart Diseases
Mary O. Gray, M.D. Professor of Medicine (Cardiology)
UC San Francisco
No Disclosures
Increased Left Ventricular Mass and Thick Walls
Hypertrophic Cardiomyopathy
Hypertension & Kidney Disease
Cardiac Amyloidosis
Mucopoly- Saccharidosis
Cardiac Sarcoidosis
Friedreich Ataxia
12/19/15
2
Endomyocardial Biopsy: Alcian Blue
Pathophysiology and Treatment of Cardiac Amyloidosis
MA Gertz, A Dispenzieri, and T Sher Nature Reviews Cardiology 12, 91-102 (2015)
12/19/15
3
Classification of Cardiac Amyloid Types
Amyloidosis Type Precursor Comment
Light Chain AL Immunoglobulin Light Chain Plasma Cell Dyscrasia
Mutant Transthyretin TTR Point Mutation Autosomal Dominant
Wild-Type Transthyretin None Senile Amyloidosis
Amyloid A Serum Amyloid A Sustained Inflammation
Isolated Atrial Amyloid Atrial Natriuretic Factor Atrial Fibrillation
12/19/15
5
Subcutaneous Fat Aspirate: Congo Red
Positive
Non-Ischemic Cardiomyopathy
Serum and Urine Immunofixation. Serum Free Light Chain Assay.
Bone Marrow Biopsy and Fat Aspiration with Congo Red Staining
AL Likely. Confirm with Mass Spectroscopy.
12/19/15
6
Diagnosis, Prognosis, and Therapy of Transthyretin Amyloidosis
MA Gertz, MD Benson, PJ Dyck, et al. JACC 2015;66:2451-2466
12/19/15
7
Transthyretin (TTR) Transporter of Thyroxine and Retinol
Positive
Non-Ischemic Cardiomyopathy
Serum and Urine Immunofixation. Serum Free Light Chain Assay.
Mass Spectroscopy and TTR Gene Sequencing
TTR Wild-Type or Mutant Fat Aspirate Endomyocardial Biopsy
Negative
12/19/15
8
Evaluation to Diagnose TTR Amyloidosis
Results Suggestive of ATTR
✔ Physical Examination Autonomic Neuropathy
✔Serum Immunofixation and
Immunoglobulin Free LC Assay LC Amyloidosis Excluded
✔ Urine Tests Nephrotic Range Proteinuria
✔Tissue and Strain Doppler
Echocardiography Diastolic Dysfunction
✔ Cardiac Magnetic Resonance Chamber Infiltration
Results Suggestive of ATTR
✔ Radionuclide Imaging Cardiac Amyloid Detection
✔ Cardiac Biomarkers Elevated Troponin Brain Natriuretic Peptide
✔ Medical History Carpal Tunnel Syndrome
✔ Tissue Biopsy: less invasive Fat and Bone Marrow
✔ Tissue Biopsy: more invasive Heart, Nerve, Kidney
✔ Mass Spectroscopy TTR Amyloid Deposits
12/19/15
9
CMR without Gadolinium: Wall Thickening
Prognostic Value of Late Gadolinium Enhancement Cardiovascular Magnetic Resonance in Cardiac Amyloidosis
M Fontana, S Pica, P Reant, et al. Circulation 2015;132:1570-1579
12/19/15
12
Heart Failure with Preserved Ejection Fraction
HFpEF Therapy and Cardiac Amyloidosis
✔ Loop Diuretic Volume Status
✔ ACE Inhibitor or ARB Blood Pressure
✔ Beta Blocker Cardiac Output
✔ Calcium Channel Blocker Cardiac Output
12/19/15
13
Arrhythmias and Cardiac Amyloidosis
✔ Amiodarone Effective
✔ Anticoagulation Effective
✔ Cardioverter-Defibrillator Possible Benefit
✔ Revascularization Possible Benefit
A European Collaborative Study of Cyclophosphamide, Bortezomib, and Dexamethasone in Upfront Treatment of Systemic AL Amyloidosis
G Palladini, S Sachchithanantham, et al. Blood 2015;126(5):612-615
12/19/15
14
AL Cardiac Stage and Survival after CyBorD
Therapeutic Clearance of Amyloid by Antibodies to Amyloid P Component
DB Richards, LM Cookson, AC Berges, et al. NEJM 2015;373:1106-1114