• Definition: a primary disease of the myocardium where there is decreased contractile
function of the myocardium, not due to pressure overload, volume overload or coronary artery disease. • Epidemiology: o Most common form of cardiomyopathy, affects middle-aged men, incidence 400,000 per year • Aetiology: o Idiopathic o ischemic heart disease o familial (~25% of idiopathic cases) o alcoholism o infective: myocarditis, post viral myocarditis o metabolic: nutritional deficiency, acromegaly, osteogenesis imperfecta, hypocalcemia, thyrotoxicosis, hypothyroidism, hypophosphatemia o familial storage disease: - glycogen storage disease, mucopolysaccharidosis o connective tissue disorders: - systemic lupus erythematosus, polyarteritis nodosa, rheumatoid arthritis, scleroderma, dermatomyositis o infiltrations and granulomas - amyloidosis, sarcoidosis, malignancy, hemochromatosis o neuromuscular - muscular dystrophy, myotonic dystrophy, Friedreich’s ataxia, o drugs/toxic reactions - radiation, cobalt, lead, phosphorus, doxorubicin, cocaine, heroin, organic solvents (glue sniffers heart) o hematologic - sickle cell anemia, thalassemia o peripartum heart disease (last trimester or 6 months postpartum) • Risk factors: alcoholism, thiamine deficiency • Pathology: o Dilatation and impaired contraction of the ventricles decrease in systolic and diastolic function increase in end-systolic and end-diastolic volumes o This leads to decrease cardiac output and increase in pulmonary venous pressure. o The heart compensates for the decreased cardiac output (CO = heart rate x stroke volume) by: increasing the heart rate and/or stroke volume, it also increased peripheral tone to maintain adequate blood pressure. This is done through: Neurohormonal activation of RAS retention of sodium & water and peripheral vasoconstriction (Stroke volume) Activation of the adrenergic nervous system increase in cardiac performance (HR) o Natriuretic peptides are elevated in dilated cardiomyopathy this is in response to volume and pressure overload in the myocardium ANP [atrial natriuretic peptide]; released from the atria, inresponse to right atrial stretch vasodilation, dieresis and inhibition of aldosterone. BNP [brain natriuretic peptide]; released from the cardiac ventricles in response to volume and pressure overload vasodilation and natriuresis • History: o Symptoms of heart failure, systemic and pulmonary embolism, arrhythmias • Examination: o Small pulse pressure o Raised JVP o Cardiomegaly, displaced apex beat, S3, functional mitral/tricuspid regurgitation, arrhythmias o Bibasal crepitations o Hepatomegaly o Hepatojugular reflux o Ankle & sacral edema • Investigations: o Bloods: FBC: low Hb (anaemia) U&E: Hyponatremia (poor prognostic factor) TFTs: TSH, T4/T3 o/r thyroid disease LFTs & Coags: deranged Alcoholism? Infiltration; Haemochromatosis?, hepatic congestion? Cardiac enzymes: to assess recent myocardial injury Urine toxicology screen: toxins? o ECG: A fib, left ventricular hypertrophy, nonspecific ST-T wave changes, LBBB? heart block? o Imaging: CXR: hypertrophy? Pulmonary vascular congestion; Kerley B lines, pleural effusion, prominent vasculature of the upper lung fields? Calcification of valves? congential malformations? ECHO: most useful diagnostic tool: • End-diastolic left ventricular dimension >65mm in dilated cardiomyopathy • Hypertrophy: defined as post. Wall or septal thickness >11mm • Doppler measurement of blood flow in the heart. Cardiovascular MRI: MRI with gadolinium-DTPA to evaluate the extent of wall fibrosis in patients with DCM and hence estimating the risk of sudden cardiac death o Invasive: Cardiac catheterisation to determine the volume status of the patient, not diagnostic Endomyocardial biopsy: indicated in patients: • With recent onset of rapidly deteriorating cardiac function • On chemotherapy with doxorubicin • Systemic disease with possible cardiac involvement (hemochromatosis, sarcoidosis, amyloidosis, etc) • Management: o Medical management as in CCF: ACE inhibitors: Enalapril 10-40mg PO OD/BD reduces mortality and improves survival (CONSENSUS trial) Beta blockers: Metoprolol 5mg initially upto 50mg BD, reduces mortality rate and improves NYHA functional class (MERIT-HF) Spironolactone [Aldosterone antagonists]; reduces mortality (RALES trial) Diuretics: Furosemide: reserved for congestive states in severe CCF or acute exacerbation of CCF. Digoxin: symptomatic relief in severe CCF o Surgical management: Partial left ventriculectomy (Batista procedure): reduce left ventricular diameter improves cardiac function. Automatic implanted cardioverter-defibrillators to treat ventricular tachycardia/fibrillation Heart transplantation: is the ultimate management of patients with DCM • Complications: o Heart failure, arrhythmias, embolism & sudden death. Hypertrophic cardiomyopathy
• Definition: A cardiomyopathy where there is asymmetrical hypertrophy of the myocardium
leading to LV out flow obstruction • Epidemiology: o 0.05-0.2% of the population, slightly more common in males, presents at younger age in females, females are more likely to be symptomatic. o Peak age: bimodal distribution: second & fourth decade. • Aetiology: o Idiopathic o familial autosomal dominant inheritance; defects in myofilament genes coding for myosin, troponin T/I, tropomyosin, cardiac actin o Subendocardial ischemia • Pathology: o Asymmetrical left ventricular hypertrophy & septal bulge LV outflow obstruction & diastolic dysfunction (impairing LV filling, etc) • History: o Sudden cardiac death most common presentation in the younger age group, this is commonly due to ventricular fibrillation (80% of cases) o Symptoms of Left heart failure: Dyspnoea: 90% of symptomatic patients; as there is LV outflow obstruction Pre-syncope & Syncope; due to LV obstruction or arrhythmias Angina: may present without coronary atherosclerosis Palpitations PND & orthopnoea Dizziness • Examination: o Double/triple apical impulses o Double carotid pulse o Systolic ejection murmur best heard between the apex beat and the left sternal border o Mitral regurgitation o 10% of patients have aortic regurgitation (diastolic murmur) • Investigations: o Bloods (non specific): FBC: low Hb (anaemia) Cardiac enzymes: to assess recent myocardial injury o ECG: A fib, left ventricular hypertrophy, nonspecific ST-T wave changes, LBBB? heart block? WPW? o Imaging: CXR: hypertrophy? Left atrial enlargement double density appearance. ECHO: most useful diagnostic tool: • Hall mark of HCM: systolic anterior motion of the anterior mitral valve leaflet and asymmetric septal hypertrophy with a ratio of septal wall thickness to posterior wall thickness of greater than 1.4:1. o Invasive: Cardiac catheterisation to determine the degree of outflow obstruction • Management: o Medical management as in CCF: Beta blockers: reduces inotropic state of left ventricle, Metoprolol 5mg initially up to 50mg BD, Calcium channel blockers: reduce inotropic state of left ventricle and decrease diastolic dysfunction. Verapamil SR 120-720 mg PO QDS Natriuretic peptides: to reduce preload and after load (dilating veins and arteries; Nesiritide 2mcg/kg IV o Surgical management: Left ventricular myomectomy: reserved for those with severe symptoms, aims to relieve LV outflow obstruction. Mitral valve replacement in severe mitral regurgitation Pacemaker implantation: AHA recommends permanent pacing for patients with HCM refractory to medical therapy Automatic implanted cardioverter-defibrillators to prevent sudden cardiac death ventricular tachycardia/fibrillation Heart transplantation: is the ultimate management of patients with DCM • Complications: o Heart failure, arrhythmias, infective mitral endocarditis, embolism & sudden death.