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Cardiomyopathy PowerPoint Presentation

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Description : Check out this medical presentation on amyloidosis, which is titled "Cardiomyopathy", to know about Cardiomyopathy, the deposition of abnormal substances (protein, cells, etc.) within the heart causing myocardial dysfunction.

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Published on : May 06, 2021 | Views : 2340 | Downloads : 2

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Slide 1-

CARDIOMYOPATHY Athena Poppas, MD Associate Professor of Medicine, Brown Medical School Director, Echocardiography Laboratory Rhode Island Hospital
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Cardiomyopathies Definition: diseases of heart muscle 1980 WHO: unknown causes Not clinically relevant 1995 WHO: “diseases of the myocardium associated with cardiac dysfunction “ pathophysiology each with multiple etiologies
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Cardiomyopathy WHO Classification anatomy & physiology of the LV Dilated Enlarged Systolic dysfunction Hypertrophic Thickened Diastolic dysfunction Restrictive Diastolic dysfunction Arrhythmogenic RV dysplasia Fibrofatty replacement Unclassified Fibroelastosis LV noncompaction Circ 93:841, 1996
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CM: Specific Etiologies Ischemic Valvular Hypertensive Inflammatory Metabolic Inherited Toxic reactions Peripartum Ischemic: thinned, scarred tissue
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Dilated Cardiomyopathy Dilation and impaired contraction of ventricles: Reduced systolic function with or without heart failure Characterized by myocyte damage Multiple etiologies with similar resultant pathophysiology Majority of cases are idiopathic incidence of idiopathic dilated CM 5-8/100,000 incidence likely higher due to mild, asymptomatic cases 3X more prevalent among males and African-Americans
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DCM: Etiology Ischemic Valvular Hypertensive Familial Idiopathic Inflammatory Infectious Viral – picornovirus, Cox B, CMV, HIV Ricketsial - Lyme Disease Parasitic - Chagas’ Disease, Toxoplasmosis Non-infectious Collagen Vascular Disease (SLE, RA) Peripartum Toxic Alcohol, Anthracyclins (adriamycin), Cocaine Metabolic Endocrine –thyroid dz, pheochromocytoma, DM, acromegaly, Nutritional Thiamine, selenium, carnitine Neuromuscular (Duchene’s Muscular Dystrophy--x-linked)
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Prognosis depends on Etiology 1230 pts. referred for unexplained CM. Felker GM. NEJM 2000;342:1077
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DCM: Infectious Acute viral myocarditis Coxasackie B or echovirus Self-limited infection in young people Mechanism?: Myocyte cell death and fibrosis Immune mediated injury BUT: No change with immunosuppressive drugs
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DCM: toxic Alcoholic cardiomyopathy Chronic use Reversible with abstinence Mechanism?: Myocyte cell death and fibrosis Directly inhibits: mitochondrial oxidative phosphorylation Fatty acid oxidation
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DCM: inherited Familial cardiomyopathy 30% of ‘idiopathic’ Inheritance patterns Autosommal dom/rec, x-linked, mitochondrial Associated phenotypes: Skeletal muscle abn, neurologic, auditory Mechanism: Abnormalities in: Energy production Contractile force generation Specific genes coding for: Myosin, actin, dystophin…
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DCM: Peripartum Diagnostic Criteria 1 mo pre, 5 mos post Echo: LV dysfunction LVEF < 45% LVEDD > 2.7 cm/m2 Epidemiology/Etiology 1:4000 women JAMA 2000;283:1183 Proposed mechanisms: Inflammatory Cytokines: TNFa, IL6, Fas/AP01 JACC 2000 35(3):701.
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PPCM: Prognosis Death from CM: ’91-97 245 CM deaths in US, 0.88/100,000 live births, 70% peripartum Increased risk with: Maternal age AA 6.4x greater Whitehead SJ. ObGyn2003;102:1326. Risk of recurrent pregnancy Retrospective survey : 44 women (16 vs 28) Reduced EF, CHF 44% vs 21%, mortality 0 vs. 19% Elkyam U. NEJM.2001;344:1567. DSE:contractile reserve reduced in patients 7 women: change in Vcfc σES relationship Lampert MB. AJOG.1997.176.189.
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Dilated Cardiomyopathy
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MECHANISMS IN HEART FAILURE Hemodynamic Derangement Clinical Heart Failure Arrhythmia Neurohormones Cytokines Oxidative stress Ischemic injury Myocardial disease Genetics Altered molecular expression Ultrastructural changes Myocyte hypertrophy Myocyte contractile dysfunction Apoptosis Fibroblast proliferation Collagen deposition Ventricular remodeling
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Pathophysiology Initial Compensation for impaired myocyte contractility: Frank-Starling mechanism Neurohumoral activation  intravascular volume Eventual decompensation ventricular remodeling myocyte death/apoptosis valvular regurgitation
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Pathophysiology: Starling Curve
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Pathophysiology: Neurohumoral Adrenergic nervous system Renin-angiotensin-aldosterone axis Vasopressin Natriuretic peptides Endothelin
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Reduced Response to Adrenergic Stimulation
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Renin-Angiotensin-Aldosterone Pathways Angiotensinogen Angiotensin-I Angiotensin-II Renin ACE AT-1 Receptor Chymase Bradykinin degradation ACE-inhibitor Angiotensin receptor blocker Aldosterone Spironolactone
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Angiotensin-II Effects Vasoconstriction Aldosterone production Myocyte hypertrophy Fibroblast proliferation Collagen deposition Apoptosis Pro-thrombotic Pro-oxidant Adrenergic stimulation Endothelial dysfunction
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The Kidney in Heart Failure Reduced renal blood flow Reduced glomerular filtration rate Increased renin production Increased tubular sodium reabsorption Increased free water retention (vasopressin)
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Ventricular Remodeling in Heart Failure
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Extracellular Stimuli of Myocyte Hypertrophy
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Clinical Findings Biventricular Congestive Heart Failure -Low forward Cardiac Output -fatigue, lightheadedness, hypotension -Pulmonary Congestion -Dyspnea, -orthopnea, & PND -Systemic Congestion -Edema -Ascites -Weight gain
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Physical Exam Decreased C.O. Tachycardia  BP and pulse pressure cool extremities (vasoconstriction) Pulsus Alternans (end-stage) Pulmonary venous congestion: rales pleural effusions Cardiac: laterally displaced PMI S3 (acutely) mitral regurgitation murmur Systemic congestion  JVD hepatosplenomegaly ascites peripheral edema
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Diagnostic Studies CXR -enlarged cardiac silhouette, vascular redistribution interstitial edema, pleural effusions EKG –normal tachycardia, atrial and ventricular enlargement, LBBB, RBBB, Q-waves Blood Tests (ANA,RF, Fe2+, TFT’s,ferritin,) Echocardiography LV size, wall thickness function valve dz, pressures Cardiac Catheterization hemodynamics LVEF angiography Endomyocardial Biopsy
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Echo in dilated CM
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Influence of EF on Survival in Patients with Heart Failure Vasan RS et al. J Am Coll Cardiol. 1999;33:1948-55
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Class 1: No limitation of physical activity. Ordinary physical activity w/o fatigue, palpitation, or dyspnea. Class 2: Slight limitation of physical activity. Comfortable at rest, but symptoms w/ ordinary physical activity Class 3: Marked limitation of physical activity. Comfortable at rest, but less than ordinary activity causes fatigue, palpitation, or dyspnea. Class 4: Unable to carry out any physical activity without discomfort. Symptoms include cardiac insufficiency at rest. If any physical activity is undertaken, discomfort is increased. J Cardiac Failure 1999;5:357-382 Criteria for NYHA Functional Classification
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Aim of Treatment Preload reduction Diuretics venodilators Vasodilators ACEI Inotropes Acutely Chronically mortality
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Vasodilator Agents in Heart Failure *Hydralazine and a long-nitrate shown to reduce mortality long-term # Other actions (aside from vasodilation) likely to be important
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Dobutamine and Milrinone Effects
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Electrical and Mechanical Ventricular Dyssynchrony Experimentally induced LBBB has effect on: expression of regional stress kinases calcium-handling proteins. Expression of p38-MAPK (a stress kinase) is elevated in the endocardium of the late-activated region, whereas phospholamban is decreased. Sarcoplasmatic reticulum Ca2+-ATPase is decreased in the region of early activation.
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Deleterious Hemodynamic Effects of LV Dyssynchrony Diminished SV & CO due: Reduced diastolic filling time1 Weakened contractility 2 Protracted MV regurgitation 2 Post systolic regional contraction 3 Atrio-ventricular Inter-V Intra-V Cazeau, et al. PACE 2003; 26[Pt. II]: 137–143 1. Grines CL, Circulation 1989;79: 845-853 2. Xiao HB, Br Heart J 1991;66: 443-447 3. Søgaard P, JACC 2002;40:723–730
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CRT: Cardiac Resynchronization Therapy 1. Improved hemodynamics Increased CO Reduced LV filling pressures Reduced sympathetic activity Increased systolic function w/o MVO2 2. Reverse LV remodeling/architecture Decreased LVES/ED volumes Increased LVEF Circ ’02, JACC ’02, JACC ’02, NEJM’02
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Risk of Sudden Death c/w EF Patients withoutLV Dysfunction (LVEF >35%) Maggioni AP. GISSI-2 Trial Circulation. 1993;87:312-322. Patients withLV Dysfunction (LVEF < 35%) No PVBs1-10 PVBs/h> 10 PVBs/h 0.86 A 0.88 0.90 0.92 0.94 0.96 0.98 1.00 0 30 60 90 120 150 180 Days Survival p log-rank 0.002 0.88 0.90 0.92 0.94 0.96 0.98 1.00 0 30 60 90 120 150 180 Days Survival B p log-rank 0.0001 0.86
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Anti-arrhythmic drugs, ICD placebo and Death
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What are the two characteristic findings in DCM?
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Hypertensive Hypertrophic Cardiomyopathy
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Women and Hypertension Prevalence of HTN in Women from NHANES-III. Burt VL. Hypertension ‘95
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Diastolic Dysfunction 40-50% of pts w/ CHF have nml LVEF Vasan JACC ’99 Grossman Circ ‘00 Prevalence: increases with age higher in women Etiology: HTN & LVH Diagnosis: MV& PV Doppler TDI, Color m-mode
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Zile MR. Circ;105:1387 Echo Doppler Parameters
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Diastolic Dysfunction Kawaguchi M. Circ 2003.107:714
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Zile MR. Circ;105:1387 Isolated Diastolic HF Isolated Systolic HF Systolic & Diastolic HF
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What is the difference between systolic and diastolic LV dysfunction?
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Hypertrophic Cardiomyopathy Left ventricular hypertrophy not due to pressure overload Hypertrpohy is variable in both severity and location: -asymmetric septal hypertrophy -symmetric (non-obstructive) -apical hypertrophy Vigorous systolic function, but impaired diastolic function impaired relaxation of ventricles elevated diastolic pressures prevalence as high as 1/500 in general population mortality in selected populations 4-6% (institutional) probably more favorable (1%)
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Etiology Familial in ~ 55% of cases with autosomal dominant transmission Mutations in one of 4 genes encoding proteins of cardiac sarcomere account for majority of familial cases -MHC cardiac troponin T myosin binding protein C -tropomyosin Remainder are spontaneous mutations.
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Hypertrophic Cardiomyopathy
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Hypertrophic Cardiomyopathy
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Hypertrophic cardiomyopathy
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Apical Hypertrophic Cardiomopathy
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Pathophysiology
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HCM with outflow obstruction Dynamic LVOT obstruction (may not be present at rest) SAM (systolic anterior motion of mitral valve) LVOT Obstruction  LVOT gradient  wall stress  MVO2  ischemia/angina  LVOT gradient:  HR (DFP), preload (LVEDV),  afterload(BP).  LVOT gradient:  BP (Afterload),  LVEDV(preload) Symptoms of dyspnea and angina more related to diastolic dysfunction than to outflow tract obstruction Syncope: LVOT obstruction (failure to increase CO during exercise or after vasodilatory stress) or arrhythmia.
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Physical Exam Bisferiens pulse (“spike and dome”) S4 gallop Crescendo/Descrescendo systolic ejection murmur HOCM vs. Valvular AS Intensity of murmur HOCM AS Valsalva (preload,  afterload)   Squatting ( preload,  afterload)   Standing (preload,  afterload)   Holosystolic apical blowing murmur of mitral regurgitation
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Diagnostic Studies EKG NSR LVH septal Q waves 2D-Echocardiography LVH; septum >1.4x free wall LVOT gradient by Doppler Systolic anterior motion of the mitral valeregurgitation Cardiac Catheterization LVOT gradient and pullback provocative maneuvers Brockenbrough phen HCM-ASH using contrast
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Cardiac Catheterization LV pullback Brockenbrough-Braunwald Sign failure of aortic pulse pressure to rise post PVC Provocative maneuvers: Valsalva amyl nitrate inhalation
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Atrial Fibrillation Acute A. Fib is poorly tolerated -Acute Pulmonary Edema and Shock Chronic a fib - Fatigue, dyspnea and angina Rapid HR - decreased time for diastolic filling and LV relaxation Loss of atrial “Kick” – decreased LV filling - decreased SV and increased outflow tract obstruction Rate slowing with -blockers and Ca2+ channel blockers Digitalis is relatively contra-indicated- positive inotrope DC Cardioversion No p wave P wave present
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Treatment For symptomatic benefit -blockers  mvO2  gradient (exercise) arrythmias Calcium Channel blockers Anti-arrhythmics afib amiodorone Disopyramide AICD for sudden death antibiotic prophylaxis for endocarditis No therapy has been shown to improve mortality
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HCM: Surgical Treatment For severe symptoms with large outflow gradient (>50mmHg) Does not prevent Sudden Cardiac Death Myomyectomy removal of small portion of upper IV septum +/- mitral valve replacement 5 year symptomatic benefit in ~ 70% of patients Dual Camber (DDD pacemaker) pacing decreases LVOT gradient (by~25%) randomized trials have shown little longterm benefit possible favorable morphologic changes ETOH septal ablation AICD to prevent sudden death
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Hypertrophic CM Most common cause of death in young people. The magnitude of left ventricular hypertrophy is directly correlated to the risk of SCD. Young pts with extreme hypertrophy and few or no symptoms are at substantial long-term risk of SCD. . Spirito P. N Engl J Med. 1997;336:775-785. Maron BJ. N Engl J Med. 2000;342:365-373.
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Wall Thickness and Sudden Death in HCM Spirito P. N Engl J Med. 2000;342:1778-1785. Maximum Left-Ventricular-Wall Thickness (mm) Incidence of Sudden Death (per 1,000 person/yr) 0 2.6 7.4 11.0 18.2
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Prognosis Sudden Death 2-4%/year in adults 4-6% in children/adolescents AICD for: survivors of SCD with Vfib episodes of Sustained VT pts with family hx of SCD in young family members High risk mutation (TnT, Arg403Gln) Predictors of adverse prognosis: early age of diagnosis familial form with SCD in 1st degree relative history of syncope ischemia presence of ventricular arrhythmias on Holter (EPS) EPS Amiodorone (low dose) Prophylactic AICD?
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HCM vs Athletes Heart Endurance training: Physiologic increase in LV mass Wall thickness and cavity size Early HCM vs Athlete’s heart DEFINITION: Symmetric, <13mm 947 elite athletes: 16 thickness=13-16mm 15 rowers, EDD=55-63 c/w 728 athletes/22 other NEJM1991;324:295 286 cyclists: 25 thickness 13-15 50% increased EDD w/ 12% reduced LVEF JACC 2004;44:144.
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Why do patients with HCM develop heart failure?
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Restrictive Cardiomyopathy Characterized by: impaired ventricular filling due to an abnormally stiff (rigid) ventricle normal systolic function (early on in disease) intraventricular pressure rises precipitously with small increases in volume Pressure Volume Causes : infiltration of myocardium by abnormal substance fibrosis or scarring of endocardium normal restriction
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Amyloid infiltrative CM
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Amyloidosis Primary Amyloidosis immunoglobulin light chains -- multiple myeloma Secondary Amyloidosis deposition of protein other than immunoglobulin senile familial chronic inflammatory process restriction caused by replacement of normal myocardial contractile elements by infiltrative interstitial deposits
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Amyloidosis
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Amyloid Cardiomyopathy
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Sarcoidosis Restriction Conduction System Disease Ventricular Arrhythmias (Sudden Cardiac Death)
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Endomyocardial Fibrosis Endemic in parts of Africa, India, South and Central America, Asia 15-25% of cardiac deaths in equatorial Africa hypereosinophilic syndrome (Loffler’s endocarditis) Thickening of basal inferior wall endocardial deposition of thrombus apical obliteration mitral regurgitation 80-90% die within 1-2 years
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Pathophysiology of Restriction Elevated systemic and pulmonary venous pressures right and left sided congestion reduced ventricular cavity size with SV and CO
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Clinical Findings Right > Left heart failure Dyspnea Orthopnea/PND Peripheral edema Ascites/Hepatomegaly Fatigue/ exercise tolerance Clinically mimics constrictive Pericarditis
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Diagnostic Studies 2D-Echo/Doppler- mitral in-flow velocity rapid early diastolic filling Catheterization – diastolic pressure equilibration restrictive vs constrictive hemodynamics Endomyocardial biopsy- definite Dx of restrictive pathology
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Cardiac Catheterization Prominent y descent “dip and plateau” rapid atrial emptying rapid ventricular filling then abrupt cessation of blood flow due to non-compliant myocardium
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Constriction vs. Restrictive CM
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Treatment Treat underlying cause r/o constriction which is treatable (restriction poor prognosis) amyloid (melphalan/prednisone/colchicine) Endomyocardial Fibrosis (steroids, cytotoxic drugs, MVR) Hemochromatosis (chelation, phlebotomy) Sarcoidosis (steroids) Diuretics For congestive symptoms, but  LV/RV filling   CO Digoxin (avoid in amyloidosis) Antiarrhythmics for afib amiodorone Pacemaker for conduction system disease Anticoagulation for thrombus (esp in atrial appendages)
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What is the hemodynamic problem in RCM?
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Arrhythmogenic RV Dysplasia Myocardium of RV free wall replaced: Fibrofatty tissue Regional wall motion/function is reduced Ventricular arrhythmias SCD in young
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MRI: RV Dysplasia
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LV Noncompaction Diagnostic Criteria Prominent trabeculations, deep recesses in LV apex Thin compact epicardium, thickened endocardium Stollberger C, JASE ‘04 Other phenotypic findings Prognosis and Treatment Increased risk of CHF, VT/SCD, thrombosis Oechslin EN, JACC ‘00 Hereditary risk Screening of offspring Pregnancy: case report
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Echo: LV Noncompaction
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Cardiomyopathy WHO Classification anatomy & physiology of the LV Dilated Enlarged Systolic dysfunction Hypertrophic Thickened Diastolic dysfunction Restrictive Myocardial stiffness Diastolic dysfunction Arrhythmogenic RV dysplasia Fibrofatty replacement Unclassified Fibroelastosis LV noncompaction