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DILATED CARDIOMYOPATHY Allen Repp, M.D. November 5, 2002 Introduction Cardiomyopathy refers to disease of the myocardium associated with cardiac dysfunction 5 types of cardiomyopathies identified by WHO, each of which may have multiple etiologies: dilated, hypertrophic, restrictive, arrhythmogenic right ventricular, and unclassified Dilated cardiomyopathy (DCM) is characterized by ventricular dilation (LV end diastolic dimension > 60 mm) and impaired contraction of one or both ventricles (LV EF generally < 30%) Idiopathic DCM is the most common primary cardiomyopathy, and the most common indication for cardiac transplantation in the US DCM is the 2nd most common cause of heart failure (after ischemic heart disease) Clinical Presentation Majority of cases occur in ages 20-60, but can occur in children or elderly Presentation may be acute, sub-acute, or chronic Symptoms may reflect numerous complications of DCM Congestive heart failure – Progressive dyspnea on exertion, impaired exercise capacity, PND, orthopnea, dependent edema, ascites, early satiety Arrhythmia – Syncope, presyncope, palpitations, sudden death Thromboembolic events – Cerebrovascular accident, pulmonary embolism Incidental detection of asymptomatic cardiomegaly Etiologies of DCM Idiopathic Cause of DCM will remain unexplained in approximately 50% after thorough evaluation Ischemic Accounts for up to 7% of cases of initially unexplained dilated cardiomyopathy Infectious Myocarditis Viral: coxsackievirus, echoviruses, adenoviruses, HIV, CMV, hepatitis, varicella, EBV Bacterial: streptococci (rheumatic fever), typhoid fever, diphtheria, brucellosis, psitticosis Spirochetal: Lyme disease (usually presents with conduction disturbances, but rarely with myocarditis and heart failure) Rickettsial disease Parasitic: Chagas disease, aka trypanosomiasis (acute tissue invasive phase or chronic phase), toxoplasmosis, shistosomiasis, trichinosis Fungal / Mycobacterial: histoplasmosis, cryptococcus Non-Infectious Myocarditis Rejection of allograft in cardiac transplant patient – the paradigm for lymphocyte-mediated myocarditis Connective tissue diseases: SLE, polymyositis Peripartum cardiomyopathy Hypersensitivity reactions Sarcoidosis – 10% of patients with sarcoidosis experience clinical cardiac involvement Giant cell myocarditis – rare, frequently fatal inflammation of myocardium with prominent ventricular tachyarrhythmias, chest pain, and hemodynamic compromise Autoimmune myocarditis – possibly related to anti-1 adrenoreceptor antibodies Celiac sprue Toxins EtOH – alcohol and metabolite acetaldehyde are direct cardiotoxins that can induce acute, reversible myocardial dysfunction or chronic, partially irreversible myocardial depression Chemotherapeutic agents – most notably, doxorubicin, but also cyclophosphamide and trastuzumab (anti-c-erbB-2 monoclonal antibody used in treatment of some breast cancers) Other medications – zidovudine, didanosine, zalcitabine, phenothiazines Heavy metals and occupational exposures – mercury, lead, beryllium, carbon monoxide Illicit drug use: amphetamines and cocaine may produce state of catecholaminergic excess Beth Israel Deaconess Medical Center Residents’ Report Radiation Metabolic Nutritional deficiencies – thiamine, selenium, carnitine Electrolyte abnormalities – hypocalcemia, hypophosphatemia, hypomagnesemia Renal failure – uremia Endocrinopathies – hypothyroidism or hyperthyroidism, growth hormone excess or deficiency, diabetes mellitus, pheochromocytoma, Cushing’s disease Familial / Inherited Neuromuscular diseases – Duchenne, Becker’s, and facioscapulohumeral dystrophy Mitochondrial myopathies – e.g. Kearns-Sayre syndrome Deposition diseases – hemochromatosis, hereditary sideroblastic anemias Other Tachycardia mediated cardiomyopathy – observed in patients with chronic supraventricular tachycardias, with severity of LV dysfunction correlating with rate of arrhythmia Obstructive sleep apnea Evaluation History Elicit onset of symptoms – rapid development over days to weeks suggests post-viral or giant cell myocarditis Characterize extent of exercise intolerance / dyspnea, as NYHA class has prognostic significance Document history of EtOH use, illicit drug use, occupational exposure history, travel history (yes, doctor, I lived in rural South America for 5 years), dietary history (especially consumption of raw meat) Family history Examination – elevated jugular venous pressure, abnormal hepatojugular reflux, hepatic distention, ascites, peripheral edema, rales, laterally displaced LV impulse, S3 or S4 gallop, RV impulse, murmurs of MR and/or TR Routine initial laboratory evaluation ECG, CXR, ECHO; CBC with differential (evaluate for eosinophilia) Serum chemistries, BUN / Cr, LFTs, CK, TSH Secondary laboratory evaluation Infectious workup – coxsackie, echovirus, influenza, HIV, Lyme, toxoplasma, trypanosoma Rheumatologic screening MRI Coronary arteriography Endomyocardial biopsy – only definite indications are monitoring transplant rejection and anthracycline toxicity Treatment Abstention from EtOH Avoidance of vigorous exercise x 3-6 months (if ongoing, active process suspected) Immunization against influenza and pneumococcus Medical therapy for CHF: ACEi, -blockers, diuretics, +/- digitalis glycosides Consider anticoagulation for patients with hx of thromboembolism, a. fib, or LV thrombus Immunosuppressive therapy with prednisone, azathioprine, or cylcosporine has been advocated by some for biopsy proven myocarditis, but there is no evidence for its efficacy at present Coronary revascularization if evidence of ischemic heart disease with myocardial viability Consideration of implantable defibrillator for patients with hx of sudden death, sustained VT, or inducible VT Cardiac transplantation Prognosis Recent onset DCM associated with 50% chance of “substantial” recovery Worse prognosis with lower functional status at presentation (1 year mortality < 10% for patients in class I CHF, but 50% for patients in class IV CHF) Outcome better in patients with peripartum cardiomyopathy, but substantially worse in patients with infiltrative disease, HIV, or doxorubicin toxicity Beth Israel Deaconess Medical Center Residents’ Report