Dilated cardiomyopathy is a primary disease of the myocardium where there is decreased contractile function not due to other causes like pressure or volume overload. It is the most common form of cardiomyopathy, affecting middle aged men. Causes include genetic factors, infections, toxins, metabolic disorders and more. Symptoms are those of heart failure. Management involves medications like ACE inhibitors, beta blockers, and diuretics, as well as procedures like ICD placement or heart transplantation. Complications include heart failure, arrhythmias and sudden death.
Hypertrophic cardiomyopathy is defined by asymmetrical hypertrophy of the myocardium leading to left ventricular outflow obstruction. It has a genetic basis and presents
Original Description:
Notes on dilated and hypertrophic cardiomyopathies.
Dilated cardiomyopathy is a primary disease of the myocardium where there is decreased contractile function not due to other causes like pressure or volume overload. It is the most common form of cardiomyopathy, affecting middle aged men. Causes include genetic factors, infections, toxins, metabolic disorders and more. Symptoms are those of heart failure. Management involves medications like ACE inhibitors, beta blockers, and diuretics, as well as procedures like ICD placement or heart transplantation. Complications include heart failure, arrhythmias and sudden death.
Hypertrophic cardiomyopathy is defined by asymmetrical hypertrophy of the myocardium leading to left ventricular outflow obstruction. It has a genetic basis and presents
Dilated cardiomyopathy is a primary disease of the myocardium where there is decreased contractile function not due to other causes like pressure or volume overload. It is the most common form of cardiomyopathy, affecting middle aged men. Causes include genetic factors, infections, toxins, metabolic disorders and more. Symptoms are those of heart failure. Management involves medications like ACE inhibitors, beta blockers, and diuretics, as well as procedures like ICD placement or heart transplantation. Complications include heart failure, arrhythmias and sudden death.
Hypertrophic cardiomyopathy is defined by asymmetrical hypertrophy of the myocardium leading to left ventricular outflow obstruction. It has a genetic basis and presents
• 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.