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dialated cardiomyopathies

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  • The threshold of left ventricular enlargement and dysfunction necessary to diagnose peripartum cardiomyopathy has not been precisely defined. The following definition, based upon a 1992 NHLBI workshop definition for idiopathic dilated cardiomyopathy, has been proposed [34,35]:
        Left ventricular ejection fraction (LVEF) less than 45 percent AND/OR M-mode fractional shortening less than 30 percent PLUS    Left ventricular end-diastolic dimension greater than 2.7 cm/m2
  • Transcript

    • 1. dilated CARDIOMYOPATHIES
    • 2. Definition  Cardiomyopathies are defined as "a heterogeneous group of diseases of the myocardium associated with mechanical and/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation and are due to a variety of causes that frequently are genetic."
    • 3. WHO Classification anatomy & physiology of the LV 1. Dilated • Enlarged • Systolic dysfunction 2. Hypertrophic • Thickened • Diastolic dysfunction 3. Restrictive • Diastolic dysfunction 3. Arrhythmogenic RV dysplasia • Fibrofatty replacement 3. Unclassified • Fibroelastosis • LV noncompaction
    • 4. Dilated Cardiomyopathy •An enlarged left ventricle with decreased systolic function as measured by left ventricular ejection fraction characterizes dilated cardiomyopathy . • Systolic failure is more marked • Diastolic dysfunction, in the setting of marked volume overload
    • 5. MajorCauses of Dilated Cardiomyopathy  Inflammatory Myocarditis – Infective/Noninfective  Toxic  Metabolic  Inherited Metabolic Pathway Defects  Familial  Idiopathic  Miscellaneous
    • 6. Pathophysiology  Brief primary injury such as infection or toxin exposure.  Some myocytes may die during the initial injury, while others survive only to have later programmed cell death, (apoptosis).  As the surviving myocytes hypertrophy to accommodate the increased burden of wall stress,  Local and circulating factors stimulate deleterious responses that contribute to progression of disease, even in the absence of further primary injury,Dynamic remodeling and the amount of ventricular dilation.  Mitral regurgitation commonly develops as the valvular apparatus is distorted by ventricular dilation
    • 7. DCM: Inflammatory Infective Reported with almost all types of infectious agents  Viral- Co xsackie , adenovirus, HIV, hepatitis C  Parasitic - T. cruzi— Chag as' dise ase , toxoplasmosis  Bacterial- diphtheria, Spirochetal ,Bo re llia  Rickettsial-Q fever  Fungal -with systemic infection
    • 8. DCM: Inflammatory Noninfective  Granulomatous inflammatory disease -Sarcoidosis ,Giant cell myocarditis  Hypersensitivity myocarditis  Polymyositis,  Dermatomyositis  Collagen vascular disease  Peripartum cardiomyopathy  Transplant rejection
    • 9. DCM: Inflammatory  Mechanism  Direct tissue injury resulting from viral infection  Immune mediated injury  Viral infection in susceptible hosts may be a proximate cause of cardiomyopathy
    • 10. DCM: Peripartum Diagnostic Criteria  1 mo pre, 6 mos post  Echo: LV dysfunction  LVEF < 45%  LVEDD > 2.7 cm/m2 Epidemiology/Etiology  1:4000 women Riskfactors - increased maternal age, increased parity, twin pregnancy, malnutrition, use of tocolytic therapy for premature labor, and preeclampsia or toxemia of pregnancyJAMA
    • 11. DCM: Peripartum Proposed mechanisms:  Inflammation - reflect increased susceptibility to viral myocarditis or  Autoimmune -myocarditis due to cross- reactivity of anti-uterine antibodies against cardiac muscle.  prolactin cleavage fragment, salt ingestion  Women with full recovery are more likely to tolerate a subsequent pregnancy than are
    • 12. DCM: Toxic  Alcohol  Catecholamines: amphetamines, cocaine  Chemotherapeutic agents: (anthracyclines, trastuzumab)  Interferon  Other therapeutic agents (hydroxychloroquine, chloroquine)  Drugs of misuse (emetine, anabolic steroids)  Heavy metals: lead, mercury  Occupational exposure: hydrocarbons, arsenicals
    • 13. DCM: Toxic Alcoholic cardiomyopathy  Toxicity is attributed both to alcohol and acetaldehyde.  Superimposed vitamin deficiencies and toxic a additives are rarely implicated.  6 drinks daily for 5–10 years, but frequent binge drinking may also be sufficient.  Reversible with abstinence  Mechanism?:  Myocyte cell death and fibrosis  Directly inhibits: mitochondrial oxidative phosphorylation Fatty acid oxidation
    • 14. DCM: Toxic Anthracyclines  Cause vacuolar degeneration and myofibrillar loss.  Generation of reactive oxygen species involving heme compounds is currently the favored explanation for myocyte injury and fibrosis.  Disruption of the large titin protein may contribute to loss of sarcomere organization.  Three different presentations  Acute heart failure/ Early onset /The chronic presentation -  Leads to a relatively nondilated ventricle, perhaps due to fibrosis.  Thus, the stroke volume may be severely reduced with an ejection fraction of 30–40%,  Therapy is suppression of "inappropriate" sinus tachycardia, and attention to postural hypotension .
    • 15. DCM: Metabolic  Nutritional deficiencies: thiamine, selenium, carnitine  Electrolyte deficiencies: calcium, phosphate, magnesium  Endocrinopathy:  Thyroid disease  Pheochromocytoma  Diabetes  Obesity  Hemochromatosis
    • 16. DCM :Familial  Skeletal and cardiac myopathy  Dystrophin-related dystrophy (Duchenne's, Becker's)  Mitochondrial myopathies (e.g., Kearns-Sayre syndrome)  Arrhythmogenic ventricular dysplasia  Hemochromatosis  Associated with other systemic diseases
    • 17. DCM :Familial  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…
    • 18. Inherited Defects in Metabolic Pathways Associated With Cardiomyopathy Glycogen Storage Diseases  II—Pompe's (alpha 1,4 glucosidase)  III—Forbes: de-branching enzyme (amylo 1,6 glucosidase) Glucose Metabolism(Defective PRKAG2a  Fatty acid metabolism  Carnitine transport defect  Medium chain Acyl-CoA dehydrogenase  Long chain Acyl-CoA dehydrogenase  Sphingolipidoses  Fabry's dise ase (alpha galactosidase A)  Gaucher disease (beta- glucocerebroside)  Disorders of lysosomal function  Danon's disease—(lysosome- associated membrane protein, LAMP2)  Miscellaneous  Hemochromatosis—Fe metabolism  Familial amyloidosis—abnormal transthyretin  Barth syndrome—tafazzin defect
    • 19. Overlap with Restrictive Cardiomyopathy  "Minimally dilated cardiomyopathy"  Hemochromatosis  Amyloidosis  Hypertrophic cardiomyopathy
    • 20. DCM: Idiopathic  Is a diagnosis of exclusion,  when all other known factors have been excluded.  two-thirds of dcm are still labeled as idiopathic;  may reflect unrecognized genetic disease.  Continued reconsideration of etiology often reveals later
    • 21. Miscellaneous Arrhythmogenic RV Dysplasia  Desmosomal complex disrupt myocyte junctions and adhesions,  Myocardium of RV free wall replaced:  Fibrofatty tissue  Regional wall motion/function is reduced  Ventricular arrhythmias  SCD in young  "woolly hair," and thickened palms and soles.
    • 22. Arrhythmogenic RV Dysplasia
    • 23. Miscellaneous LV Noncompaction Diagnostic Criteria  Prominent trabeculations, deep recesses in LV apex  Thin compact epicardium, thickened endocardium  associated with multiple genetic variants in the sarcomeric and other proteins such as tafazzin Prognosis and Treatment  Increased risk of CHF, VT/SCD, thrombosis  Hereditary risk  Screening of offspring
    • 24. Echo: LV Noncompaction
    • 25. Miscellaneous Tako-Tsubo Cardiomyopathy  The apical ballooning syndrome, or stress-induced cardiomyopathy, occurs typically  In older women after sudden intense emotional or physical stress  Presentations include pulmonary edema, hypotension, and chest pain with ECG changes mimicking an acute infarction.  May result from intense sympathetic activation with heterogeneity of myocardial autonomic innervation, diffuse microvascular spasm, and/or direct catecholamine toxicity.
    • 26. Tako-Tsubo Cardiomyopathy
    • 27. Evaluation of the DCM HISTORY  Detailed family history  History of alcohol, illicit drugs, chemotherapy or radiation therapy  A past or associated history of rheumatologic, endocrine, or infectious diseases  Assessment of ability to perform routine and desired activities  Assessment of volume status, orthostatic blood pressure, body mass index
    • 28. 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 mitral regurgitation murmur Systemic congestion ↑ JVD hepatosplenomegaly ascites peripheral edema
    • 29. Chemistry  Serum sodium, potassium,calcium,magnesiu m  Fasting glucose  Creatinine,blood urea nitrogen  Albumin, total protein,  Liver function tests  Lipid profile  Thyroid-stimulating hormone  Serum iron, transferrin saturation Hematology:  Hemoglobin/hematocrit  White blood cell count with differential,including eosinophils  Erythrocyte sedimentation rate
    • 30. SEROLOGY  Acute viral (coxsackie virus, echovirus, influenza virus)  Human immunodeficiency virus,  Chagas' disease,  Lyme disease,  Toxoplasmosis
    • 31. X-RAY CHEST  Cardiomegaly  Pulmonary vascular congestion  Kerley B lines  Prominent vasculature of the upper lung fields.  Pleural effusion usually on the right side, but it can be bilateral
    • 32. Electrocardiogram  No specific electrocardiographic findings  Sinus tachycardia is often present  poor R wave progression, intraventricular conduction delays, and LBBB.  A wide QRS complex portends a worse prognosis  left ventricular fibrosis may exhibit anterior Q waves  nonspecific ST-segment and T wave abnormalities as well as P wave alterations  Persistent supraventricular or ventricular tachyarrhythmias represent an important etiologic factor for ventricular dysfunction
    • 33. ECHOCARDIOGRAPHY.  Cornerstone in the evaluation and management  LVEDD are usually greater than 60 mm  Global hypokinesia  Decreased EF and FS  Associated ds
    • 34. ECHOCARDIOGRAPHY
    • 35.  CARDIAC MRI AND MULTIDETECTOR CT  RADIONUCLIDE IMAGING  INVASIVE EVALUATION INCLUDING  ENDOMYOCARDIAL BIOPSY.
    • 36. Management PHARMACOLOGIC ANDDEVICE THERAPY  Neurohormonal antagonists to prevent disease progression  Diuretics to maintain the volume balance are the therapeutic cornerstone  Prophylactic implantable cardiac defibrillators and biventricular pacemakers is indicated in appropriate patients
    • 37. 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
    • 38. SURGERY.  Patients with structural heart ds conditions corrected  Left ventricular assist devices- provide aggressive mechanical support to patients with advanced decompensated heart failure EMERGING SPECIFIC THERAPIES  infections and immunomodulatory agents  Stem cells for cardiac regeneration and gene
    • 39. Presentation with Symptomatic Cardiomyopathy Dilated Restrictive Hypertrophic Ejection fraction (normal 55%) Usually <30% 25-50% >60% LVDD (nor<55 mm) 60 mm >60 mm (may be decreased) Often decreased Left ventricular wall thickness Decreased Normal or increased Markedly increased Atrial size Increased Increased; may be massive Increased; related to abnormal Valvular regurgitation Related to annular dilation; mitral earlier, during decompensation; tricuspid late stages Related to endocardial involvement; frequent mitral and tricuspid regurgitation, rarely severe Related to valve- septum interaction; mitral regurgitation Common first symptoms Exertional intolerance Exertional intolerance, fluid retention early Exertional intolerance; may have chest pain
    • 40. THANKS !