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Cardiomyopathy and Myocardial Disease

Three Broad Categories of Cardiomyopathy. Dilated*HypertrophicRestrictive. Definition and classification of cardiomyopathy- American Heart Association (AHA) in 2006. Mechanical and/or electrical dysfunction that usually exhibit inappropriate ventricular hypertrophy or dilitationDue to a variety

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Cardiomyopathy and Myocardial Disease

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    1. Cardiomyopathy and Myocardial Disease Jill R. Tichy M.D. PGY II

    2. Three Broad Categories of Cardiomyopathy Dilated* Hypertrophic Restrictive

    3. Definition and classification of cardiomyopathy- American Heart Association (AHA) in 2006 Mechanical and/or electrical dysfunction that usually exhibit inappropriate ventricular hypertrophy or dilitation Due to a variety of causes; frequently genetic Either confined to the heart or are a part of generalized systemic disorders

    4. Cardiomyopathy Semantics courtesy of AHA and ESC Originally defined as disorders that are idiopathic/genetic Valvular, Ischemic or Hypertensive cardiomyopathy was deemed incongruent with the above definitions in 2006-2007

    5. Dilated Cardiomyopathy (DCM) by definition Chamber enlargement and impaired systolic function of one or both ventricles Normal LV wall thickness Unrelated to hypertension or ischemic heart disease

    6. DCM related facts Estimated prevalence of 1:2500 Third most common cause of heart failure Most frequent cause of heart transplantation

    7. A word on myocarditis Inflammation of the myocardium Frequently a result from viral infections-direct invasion into the myocytes During acute phase of illness cardiac enzyme leakage is found Endomyocardial biopsy of the right ventricle remains the gold standard for diagnosis

    8. Myocarditis Clinical course variable In some patients depressed ventricular function may develop in the absence of symptoms Manifests months to years later as dilated cardiomyopathy

    9. Causes of DCM Idiopathic Toxin induced: EtOH, Anthracycline, Cobalt, Cocaine, Crystal Meth Radiation Infectious: Viral (coxsackievirus, adenovirus, parvovirus, human immunodeficiency virus [HIV]) , Parasites (Chagas’ Disease) High Ouput States: Anemia, Peripartum, Thiamine deficiency, Thyrotoxicosis Sarcoidosis Amyloidosis Hemochromatosis Genetic

    10. Idiopathic DCM-diagnosis of exclusion Responsible for half of DCM diagnoses Exclusion of > 50% obstruction of one or more coronary arteries Exclusion of active myocarditis Exclusion of a primary or secondary form of heart muscle disease

    11. Genetic causes of DCM About 20-30% of dilated cardiomyopathy cases have been reported as familial Autosomal Dominant trait with variable penetrance

    12. Dilated (Congestive) DCM Symptoms Dyspnea on exertion Orthopnea, paroxysmal nocturnal dyspnea Fatigue Lower Extremity Edema, increasing abdominal girth

    13. Sequelae Ventricular and supraventricular arrhythmias Conduction system abnormalities Thromboembolism Sudden or heart failure–related death.

    14. Medical Management of DCM Similar to congestive heart failure Clinical outcome has improved with ACEI and more recently B-Blocker therapy Supportive: avoidance of exercise, EKG monitoring for arrhythmias, contraception?

    15. Prognosis of advanced heart failure in DCM 5-year mortality is in excess of 50% Hyponatremia at the time of presentation was found to be a marker of increased stimulation of the renin-angiotensin axis and of worsening of the disease course and prognosis.

    16. References Andreoli, et al. Cecil Essentials of Medicine. 6th Edition. Griffin, B. Manual of Cardiovascular Medicine. 2004 Cooper,L. “ Definition and classification of the cardiomyopathies, a literature review”. Feb 2008 Blackstock, U. “Cardiomyopathy, Dilated-a literature review”. Dec 2007

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