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Definition of cardiomyopathies
Cardiomyopathy can be broken down into three terms: Cardio – referring to the heart, “myo” –
indicating muscle of which the heart mainly consist and pathology – referring to disease. Therefore
Cardiomyopathy refers to a disease which affects the heart muscle. This condition can affect
anyone of any age group or ethnicity and is not curable, but can be treated.
Aetiology
There are many different forms or Cardiomyopathy; the three most common forms are
Hypertrophic Cardiomyopathy (HCM), Dilated Cardiomyopathy (DCM) and Arrhythmogenic right
ventricular Cardiomyopathy (ARVCM).
Hypertrophic Cardiomyopathy is caused when a portion of the heart muscle becomes thickened it
has no known cause and is the Cardiomyopathy known to cause sudden unexpected death.
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Dilated Cardiomyopathy is when the heart muscle becomes weakened and unable to pump
efficiently there is no definite cause of DCM but certain studies have shown that alcohol abuse and
exposure to toxins can lead to this form of Cardiomyopathy.
Arrhythmogenic Right Ventricular Cardiomyopathy is caused by a genetic defect in the proteins of
the heart muscle, specifically those that make up the desmesomes of the myocardium, this causes
arrhythmias in the wall of the right ventricle.
Pathophysiology
Cardiomyopathies constitute a group of diseases in which the dominant feature is involvement of
the heart muscle. The cause is often unknown or unclear, but may be identified with a primary or
secondary process.
Primary cardiomyopathy
• Involvement of the myocardium alone.
• Cause is known or unknown (idiopathic).
Secondary cardiomyopathy
• Associated with the dysfunction of other organ systems.
• May develop secondary to an increase of pressure and fluid overload.
•
Compensatory hypertrophy mechanisms eventually cause degenerative changes in cardiac
muscle.
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Functional classification
1) Dilated Cardiomyopathy
• Myocardium is pale with areas of fibrosis but no significant hypertrophy.
• Heart is globular with poor contraction & low ejection fraction.
• See markedly dilated left ventricle, low cardiac output and poor systolic function.
2) Hypertrophic Cardiomyopathy
• See inappropriate myocardial hypertrophy, often involving interventricular septum
of a non dilated left ventricle.
• Diastolic dysfunction: abnormal stiffness of left ventricle during diastole->
impaired ventricular filling -> increased left ventricular filling pressures ->
pulmonary congestion.
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3) Restrictive Cardiomyopathy
• Myocardium & endocardium are covered with fibroelastic tissue.
• See abnormal ventricular compliance & increased ventricular diastolic pressures.
• Fibro elastic tissue results in a rigid heart that does not distend well during. diastole
nor completely contract in systole.
• Results in low cardiac output and congestive heart failure
• May resemble restrictive pericarditis.
Signs and symptoms
• Can mimic those of almost any form of heart disease.
• Chest pain is most common.
• Mild cases are frequently asymptomatic.
• Severe cases are associated with heart failure, arrhythmias, and systematic embolization.
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• ECG abnormalities are present. Left ventricular hypertrophy results in flat or inverted T
waves.
Medical management
The main goals of treating cardiomyopathy include:
• Managing any conditions that cause or contribute to the disease.
• Controlling signs and symptoms so that you can live as normally as possible.
• Stopping the disease from getting worse.
• Reducing complications and the risk of sudden cardiac arrest.
Physiotherapy management
Phases of recovery
Phase: 1 - the time of inpatient management.
Phase: 2 - outpatient recovery.
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Phase: 3 -long-term maintenance.
Management
• Minimizing the effects of bed rest.
• Mobilization of the patient as soon as possible.
• Enhancing cardiovascular and musculoskeletal functioning.
• Encouraging attendance at an outpatient program /exercise class.
• Relaxation training (relaxed breathing positions, diaphragmatic breathing).
• Exercise testing (exercise tolerance, cardio-respiratory endurance)
• Investigation of work environment.
• Health promotion/education with regard to life style.