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Transcript
Cardiomyopathy Disorder of heart muscle 1. Idiopathic Congestive (Dilated) Cardiomyopathy (Increased ventricle size, decreased contractility) Prevalence: 0.2% Mortality: 40% in 2yrs (sudden death, cardiogenic shock) Typical Patient Young man RVF LVF Cardiomegaly AF +/- emboli Exclude - Ischaemia, Valvular disease, High BP Pericardial disease Specific heart muscle diseases Echocardiography - globally hypokinetic, dilated heart Other tests: no or non-specific abnormalities Management: as for heart failure. Consider transplant. 2. Hypertrophic (Obstructive) Cardiomyopathy Hypertrophy of ventricular muscle of no known cause. Leads to: Gradually falling LV function Sudden death Risk increased by: Hard exercise Anaesthesia Family history of sudden death Paroxysmal AF Ventricular tachycardia Prevalence: 0.2% Associations 25% associated with subaortic obstruction and/or systolic anterior motion of mitral valve 70% associated with gene mutations Inheritance: Autosomal dominant (50% are sporadic) Screening relatives: Helpful preventative measures, but life insurance problems Symptoms Often no Dyspnoea (PND) Exertional or atypical chest pain Faints (15-25%, due to arrhythmias) Palpitations Features of right heart failure Signs Often none Jerky pulse (rapid upstroke) Double impulse at apex S3 S4 Late systolic murmur (outflow tract obstruction +/- mitral regurge) AF (in ~5%) ECG: LVH, LBBB Echocardiography: Usually diagnostic Asymmetrical septal hypertrophy Systolic anterior movement of mitral valve Midsystolic closure of aortic valve Cardiac Catheterisation: Small banana-like LV cavity Thickened papillary muscles and trabeculae Cavity obliteration in systole Management ß-blockers or Verapamil for chest pain Amiodarone for arrhythmias Try to maintain sinus rhythm Dual chamber pacing - in outflow obstruction Consider septal myomectomy Paroxysmal AF - anticoagulate 3. Restrictive Cardiomyopathy Endomyocardial stiffening Signs as in Constrictive Pericarditis Commonest in the Tropics (due to Idiopathic fibrosis) In UK commonest cause - Amyloidosis Differential Diagnosis - specific muscle diseases Amyloid and Carcinoid may be restrictive (e.g. Amyloid and cardiac involvement in Friedreich's ataxia - may resemble HOCM) Chief causes - Ischaemia, Hypertension, Infection (Rheumatic fever, Infective Endocarditis, Tuberculosis, Lyme disease), Alcohol, Cocaine, Ecstasy, Post-partum, smoking, connective tissue diseases, diabetes, hyper- or hypothyroidism, Acromegaly, Addison's disease, Phaeochromocytoma, Haemochromatosis, Sarcoid, Duchenne Muscular Dystrophy, myotonic dystrophy, irradiation, cytotoxics, storage diseases. ACUTE MYOCARDITIS Inflammation of the myocardium May present similarly to MI Causes: Viral Coxsackie Lyme disease Diptheria Other infections Rheumatic fever Drugs The Patient: Faints Rapid pulse Angina Dyspnoea Arrhythmia Heart Failure Tests: Exclude MI and pericardial effusion Consider viral or Chagas' serology Management: Supportive May recover spontaneously or may develop intractable heart failure