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    Add as FriendCardiomyopathy

    by: RAJ

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    1 : Cardiomyopathies M. Devarasu Stanley Medical College Chennai-600 001 Tamil Nadu, South India.
    2 : Definition “A primary disorder of the heart muscle that causes abnormal myocardial performance and is not the result of disease or dysfunction of other cardiac structures … myocardial infarction, systemic hypertension, valvular stenosis or regurgitation”
    3 : Classification etiology gross anatomy histology genetics biochemistry immunology hemodynamics functional prognosis treatment
    4 : Functional Classification Dilatated (congestive, DCM, IDC) ventricular enlargement and syst dysfunction Hypertrophic (IHSS, HCM, HOCM) inappropriate myocardial hypertrophy in the absence of HTN or aortic stenosis Restrictive (infiltrative) abnormal filling and diastolic function
    5 : Idiopathic Dilated Cardiomyopathy a disease of unknown etiology that principally affects the myocardium LV dilatation and systolic dysfunction pathology increased heart size and weight ventricular dilatation, normal wall thickness heart dysfunction out of portion to fibrosis
    6 : Predicting Prognosis in IDC Predictive Possible Not Predictive Clinical factors symptoms alcoholism age peripartum duration family history viral illness Hemodynamics LVEF LV size Cardiac index atrial pressure Dysarrhythmia LV cond delay AV block simple VPC complex VPC atrial fibrillation Histology myofibril volume other findings Neuroendocrine hyponatremia plasma norepinephrine atrial natriuretic factor
    7 : Clinical Manifestations Highest incidence in middle age blacks 2x more frequent than whites men 3x more frequent than women symptoms may be gradual in onset acute presentation misdiagnosed as viral URI in young adults uncommon to find specific myocardial disease on endomyocardial biopsy
    8 : History and Physical Examination Symptoms of heart failure pulmonary congestion (left HF) dyspnea (rest, exertional, nocturnal), orthpnea systemic congestion (right HF) edema, nausea, abdominal pain, nocturia low cardiac output fatigue and weakness hypotension, tachycardia, tachypnea, JVD
    9 : Cardiac Imaging Chest radiogram Electrocardiogram 24-hour ambulatory ECG (Holter) lightheadedness, palpitation, syncope Two-dimensional echocardiogram Radionuclide ventriculography Cardiac catheterization age >40, ischemic history, high risk profile, abnormal ECG
    10 : Clinical Indications for Endomyocardial Biopsy Definite monitoring of cardiac allograft rejection monitoring of anthracycline cardiotoxicity Possible detection and monitoring of myocarditis diagnosis of secondary cardiomyopathies differentiation between restrictive and constrictive heart disease
    11 : Pathophysiology Systole dynamic outflow tract gradient Diastole impaired diastolic filling, ? filling pressure Myocardial ischemia ? muscle mass, filling pressure, O2 demand ? vasodilator reserve, capillary density abnormal intramural coronary arteries systolic compression of arteries
    12 : Clinical Manifestation Asymptomatic, echocardiographic finding Symptomatic dyspnea in 90% angina pectoris in 75% fatigue, pre-syncope, syncope ? risk of SCD in children and adolescents palpitation, PND, CHF, dizziness less frequent
    13 : Risk Factors for SCD Young age (<30 years) “Malignant” family history of sudden death Gene mutations prone to SCD (ex. Arg403Gln) Aborted sudden cardiac death Sustained VT or SVT Recurrent syncope in the young Nonsustained VT (Holter Monitoring) Brady arrhythmias (occult conduction disease) Br Heart J 1994; 72:S13
    14 : Management beta-adrenergic blockers calcium antagonist disopyramide amiodarone, sotolol DDD pacing myotomy-myectomy plication of the anterior mitral leaflet
    15 : Other Causes of Hypertrophy Clinical mimics glycogen storage, infants of diabetic mothers, amyloid Genetic Noonan’s, Friedreich’s ataxia, Familial restrictive cardiomyopathy with disarray Exaggerated physiologic response Afro-Caribbean hypertension, old age hypertrophy, athlete’s heart
    16 : Hypertensive HCM of the Elderly Characteristics modest concentric LV hypertrophy (<22 mm) small LV cavity size associated hypertension ventricular morphology greatly distorted with reduced outflow tract sigmoid septum and “grandma SAM”
    17 : Restrictive Cardiomyopathies Hallmark: abnormal diastolic function rigid ventricular wall with impaired ventricular filling bear some functional resemblance to constrictive pericarditis importance lies in its differentiation from operable constrictive pericarditis
    18 : Clinical Manifestations Symptoms of right and left heart failure Jugular Venous Pulse prominent x and y descents Echo-Doppler abnormal mitral inflow pattern prominent E wave (rapid diastolic filling) reduced deceleration time (? LA pressure)
    19 : Restriction vs Constriction History provide can important clues Constrictive pericarditis history of TB, trauma, pericarditis, sollagen vascular disorders Restrictive cardiomyopathy amyloidosis, hemochromatosis Mixed mediastinal radiation, cardiac surgery
    20 : Treatment No satisfactory medical therapy Drug therapy must be used with caution diuretics for extremely high filling prssures vasodilators may decrease filling pressure ? Calcium channel blockers to improve diastolic compliance digitalis and other inotropic agents are not indicated
    21 : Thank you for your attention

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