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Overview of Cardiac Amyloidosis PowerPoint Presentation

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Description : Check out this medical presentation on amyloidosis, which is titled "Overview of Cardiac Amyloidosis", to know about the infiltrative cardiomyopathy and its causes.

Tags : amyloidosis, Overview, Cardiac Amyloidosis, infiltrative cardiomyopathy, causes, Amyloidosis, Sarcoidosis

Published on : May 06, 2021 | Views : 5059 | Downloads : 45

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Slide 1-

Overview of Cardiac Amyloidosis Farooq Sheikh, M.D., FACC Assistant Professor of Medicine, Georgetown University Director, Infiltrative Cardiomyopathy Program
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Case #1 63 year old African American man presents with exertional dyspnea and fatigue. Past Medical History: HTN, T2DM with peripheral neuropathy, obesity, chronic back pain, history of bilateral carpal tunnel release, family history of “heart problems”. Recently diagnosed with “bone marrow problem”. Objective data: ECG: sinus rhythm at 68 bpm with 1st degree AV block. Computer states “low voltage ECG”.
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Case #1 Objective data (continued): Echo: LVEF 45%, moderate to severe concentric LVH with IVS thickness of 1.5-1.6 cm. Mild MR and TR. Mild RV systolic dysfunction. Physical exam: +JVD, 1/6 HSM, 1+ LE edema. Labs: Sodium 133, Cr 1.5, Free light chain assay K/L ratio 2.6. Serum immunofixation: kappa “spike” detected. Cardiac amyloidosis is suspected. How do you evaluate?
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Infiltrative Cardiomyopathy Characterized by the deposition of abnormal substances (protein, cells, etc.) within the heart causing myocardial dysfunction Diagnosis requires a high degree of suspicion Imaging and laboratory testing are essential Represents cardiac manifestation of a systemic disease
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Causes of Infiltrative CMs Amyloidosis Sarcoidosis (and other inflammatory diseases) Hemochromatosis Fabry disease (lysosomal storage) Danon disease (LAMP2 deficiency) PRKAG2 deficiency Friedreich ataxia (frataxin mutation) Hypereosinophilic syndromes Carcinoid
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Amyloidosis Systemic process resulting in extracellular deposition of abnormal insoluble fibrils Derived from a variety of serum proteins Can affect one or more organs Results in organ dysfunction due to tissue infiltration Falk RH, Circulation 2005, 2011
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Amyloidosis is a Multisystem Disease CNS MANIFESTATIONS Progressive dementia Headache Ataxia Seizures Spastic paresis Stroke-like episodes AUTONOMIC NEUROPATHY Orthostatic hypotension Recurrent urinary tract infection (due to urinary retention) Sexual dysfunction Sweating abnormalities CV MANIFESTATIONS Conduction blocks Cardiomyopathy/cardiac hypertrophy Systolic and/or diastolic dysfunction Atrial and/or ventricular arrhythmias Mild valvular regurgitation Shortness of breath Fluid retention (edema, ascites) PERIPHERAL SENSORY MOTOR NEUROPATHY Neuropathic pain Altered sensation (i.e., change in sensitivity to pain and temperature) Numbness and paresthesia Muscle weakness Impaired balance Difficulty walking RENAL Proteinuria Renal failure CARPAL TUNNEL SYNDROME OCULAR MANIFESTATIONS Vitreous opacification Glaucoma Abnormal conjunctival vessels Papillary abnormalities GI MANIFESTATIONS Nausea and vomiting Changes in GI motility (i.e., diarrhea, constipation, gastroparesis, early satiety) Unintentional weight loss Conceição et al. J Peripher Nerv Syst 2016;21:5–9
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Cardiac Amyloidosis Presentations Heart failure (dyspnea, edema, etc) Angina Syncope/presyncope (conduction disease) Sudden death Unexplained “hypertrophy” ECG/Echo abnormalities Pericardial disease Thromboembolism/stroke Falk RH, Prog Cardiovasc Dis, 2010
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Clues for Cardiac Amyloidosis Shah KB et al., Circ HF 2016
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Diagnostic Evaluation: Precursor Protein Falk RH et al., JACC 2016
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Slide courtesy of Lowell Satler
Slide 12-

AL Cardiac AmyloidImmunoglobulin Molecule
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Giampaolo Merlini, M.D. (Kyoto Myeloma Conference, 2013)
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Transthyretin (TTR) Cardiac Amyloidosis There are 2 forms of disease: Wild type amyloidosis or senile systemic amyloidosis (ATTRwt) Familial (Hereditary) amyloid cardiomyopathy (hATTR or ATTRm) Ruberg FL, Berk JL, Circulation 2012
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ATTR Amyloidosis 1. Hawkins et al. Ann Med 2015;47:625–38; 2. Hanna. Curr Heart Fail Rep 2014;11:50–7; 3. Damy et al. J Cardiovasc Transl Res 2015;8:117–27
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THAOS Registry: Distribution of ATTR Mutations Maurer MS et al., Circulation 2017
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ATTR Disease and Aortic Stenosis Castano A et al., European Heart Journal 2017
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ATTR Disease and HFpEF Mohammed SF et al., JACC HF 2014
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Cumulative Mortality (WT vs. Val122Ile) Maurer MS et al., JACC 2016
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Diagnosis Requires Pattern Recognition Hanna M, Cleveland Clinic Journal of Medicine, 2017
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Diagnostic Tools: Electrocardiogram Falk RH, Circulation, 2011
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Echocardiography
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Echo Strain Imaging Falk RH et al., JACC 2016
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Cardiac MRI Esplin BL, Curr Probl Cardiol, 2013
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Technetium Pyrophosphate Imaging Bokhari S, J Nucl Cardiol, 2014
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Slide courtesy of Lowell Satler
Slide 27-

Proteomic Analysis of Biopsy Tissue Vrana JA et al, Blood 2009
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May 7, 2021 ‹#›
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General Principles for Treatment of Cardiac Amyloidosis Treatment depends on the identification of the precursor protein Diuretics/sodium restriction Surveillance for and treatment of atrial/ventricular arrhythmias Monitoring for conduction disease Avoidance of negative inotropic/chronotropic drugs (beta blockers, calcium channel blockers) Avoidance of vasodilator therapy
Slide 30-

Multidisciplinary Team Approach
Slide 31-

Treatment Strategies in TTR Disease 1. Hawkins et al. Ann Med 2015;47:625–38; 2. Hanna. Curr Heart Fail Rep 2014;11:50–7; 3. Damy et al. J Cardiovasc Transl Res 2015;8:117–27
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Current and Emerging Therapies for ATTR Amyloidosis Buxbaum JN, NEJM 2018
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Slide courtesy of Lowell Satler
Slide 34-

Tafamidis: ATTR-ACT Trial Maurer MS et al, NEJM 2018 FDA-approved for treatment of ATTR cardiac amyloidosis
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ATTR-ACT Trial NNT to prevent 1 death=7.5 NNT to prevent 1 hospitalization per year=4.5 Maurer MS et al, NEJM 2018
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ATTR-ACT Trial: 6MWT Distance Maurer MS et al, NEJM 2018
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Tafamidis Survival by NYHA Class Maurer MS et al, NEJM 2018
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Current and Emerging Therapies for hATTR Amyloidosis Buxbaum JN, NEJM 2018
Slide 39-

Patisiran (APOLLO Trial) Adams D et al, NEJM 2018 FDA-approved for treatment of hATTR polyneuropathy
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Patisiran:Effects on Cardiac Parameters Solomon SD et al, Circulation 2018
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Inotersen: NEURO-TTR Trial Benson M.D. et al, NEJM 2018 FDA-approved for treatment of hATTR polyneuropathy
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Biomarker Staging System in Wild Type Cardiac Amyloidosis (TnT and NT-proBNP) Grogan M et al., JACC 2016
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Diagnosis of ATTR Amyloidosis is Often Delayed 1. Adams et al. Amyloid 2012;19(Suppl. 1):61–4; 2. Plante-Bordeneuve et al. Neurology 2007;69:693–8; 3. Cappellari et al. J Periph Nerv Syst 2011;16:119–29; 4. Koike et al. J Neurol Neurosurg Psychiatry 2012;83:152–8; 5. Leibou et al. Isr Med Assoc J 2012;14:662–5; 6. Luigetti et al. Neurol Sci 2013;34:1057–63 (n=60) Adams1 (n=15) Luigetti6 (n=17) Cappellari3 (n=50) Koike4 (n=90) Plante-Bordeneuve2 (n=8) Leibou5 Mean Delay in Diagnosis of hATTR Amyloidosis (Years) 3.1 4.3 3.3 2.8 2.0 4.0
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ATTR Amyloidosis: Delay from Symptom Onset to Diagnosis Early and common manifestation Papoutsidakis et al. J Card Fail 2018;24:131–3
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AL Cardiac Amyloidosis Treatment Chemotherapy is the cornerstone therapy Hanna M, Cleveland Clinic Journal of Medicine, 2017
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Treatment and Prognosis in AL Amyloid Maurer MS et al., Circulation 2017
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Conclusions Cardiac amyloidosis (CA) results from extracellular deposition of abnormal insoluble fibrils (immunoglobulin light chain or transthyretin). Disease recognition requires a high degree of suspicion (“pattern recognition”). Early diagnosis is the key to improving survival. TTR CA can now be diagnosed without the need for biopsy given improvements in non-invasive techniques. There are FDA-approved treatments for AL and TTR CA that improve survival and QOL.
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MHVI Infiltrative Cardiomyopathy Program Within the section of Advanced Heart Failure Currently focused on amyloidosis and sarcoidosis Farooq Sheikh Selma Mohammed Ajay Kadakkal