Dilated Cardiomyopathy ICM-9CM Code: 425.

4 Other Primary Cardiomyopathies Definition : The Heart Is Enlarged & Both Ventricles Are Dilated 1. Epidemiology: A. Increases With Age B. Approaches 10% At Age 80 Years 2. Etiology: A. Idiopathic B. Alcoholism D. Collagen Vascular Disease 1. SLE 2. Rheumatoid Arthritis 3. Polyarteritis Nodosa 4. Dermatomyositis E. Inherited Form: 1. Related to Becker & Duchenne Muscular Dystrophy A. Dystrophic Gene On The X Chromosome B. Other Cytoskeletal Proteins Involved: 1. Desmin 2. Sarcoglycan & Merosin : Dystrophin Related Proteins F. Peripartum Cardiomyopathy G. Nutritional Deficiencies: 1. Beri Beri 2. Thiamine Deficiency, H. Selenium Deficiency , Carnitine Deficiency I. Heroin, Cocaine, Organic Solvents J. Radiation K. Endocrine Causes: 1. Acromegaly 2. Diabetes Mellitus 3. Thyrotoxicosis 4. Myxedema

M. Toxin Induced: 1. Co, Pb, Hg N. Hypocalcemia O. Postmyocarditis P. Infections: 1. HIV 2. Rickettsial 3. Mycovacteria 4. Toxoplasmosis 5. Trichinosis 6. Chagas Disease Q. Drug Induced: 1. Phenothiazines 2. Doxorubicin 3. Antiretroviral Agents 1. Zidoviudine 2. Didanosine 3. ZalcitabineR R. Hematologic States: 1. Sickle Cell Anemia 3. Clinical Features A. Dyspnea on Exertion B. Orthopnea C. Paroxysmal Nocturnal Dyspnea D. Palpitations E. Systemic and Pulmonary Embolism

4. Physical Findings: 1. Increased JVP 2. Small Pulse Pressure 3. Pulmonary Rales 4. Hepatomegaly 5. Peripheral Edema 6. S3, S4 7. Mitral Regurgitation 8. Tricuspid Regurgtation A. Ineffective Contractility of the Heart B. Ejection Fraction Less Than 25% (Normal 50-65%) C. Progressive CHF 5. Work Up: A. History and Physical Examination B. Chest X-Ray C. EKG 1. Left Ventricular Hypertrophy With 2. ST-T Wave Changes 3. Arrhythmias: A. Atrial Fibrillation B. PVCs C. Premature Atrial Contractions D. Ventricular Tachycardia D. Echocardiography. E. Cardiac Troponin Levels: 1. Persistently Elevated Troponin T Levels: Marker For Prognsis

6. Imaging Studies: A. Chest X-Ray: 1. Massive Cardiac Enlargement 2. Interstitial Pulmonary Edema B. Echocardiography: 1. Low Ejection Fraction With 2. Global Akinesia 7. General Management A. Treat CHF: 1. Salt Restriction 2. Diuretics 3. ACE Inhibitors 4. Beta-Blockers A. Carvedilol 5. Digitalis 6. Spironolactone B. Left Ventricular Dysfunction: 1. Vasodilators 2. Nitrates 3. ACE Inhibitors C. Oral Anticoagulation: 1. For Atrial Fibrillation 2. Moderate To Severe Heart Failure D. Anti-Arrhythmic Treatment 1. Where Appropriate E. Surgical Revascularization 1. LVEF<25% & 2. Associated Atherosclerosis: A. Angina B. EKG Changes C. Reversible Defects On Thallium Scan F. Cardioverter-Defibrillators 1. With Severe -Non-Ischemic Dilated Cardiomyopathy

8. Course of Disease: Death From: A. Intractable Heart Failure B. Embolic Complications C. Ventricular Arrythymias 9. Mortality: A. Annual Mortality 1. 20% In Patients With Moderate Heart Failure 2. >50% In Patients With Severe Heart Failure 10. Referral: A. Comsider Heart Transplant 1. Young Patients < 60 Years of Age Who Are No Longer Responsive to Medical Therapy 11. Differential Diagnosis: A. Pulmonary Diseases B Valvular Dysfunction C. Pericardial Abnormalities D. Coronary Atherosclerosis E. Psychogenic Dyspnea