Cardiomyopathy

Dilated cardiomyopathy:

• 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.