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CARDIOMYOPATHY
- JOISY ALOOR
LEONARD SHAJU
SMIT BHAISARE
SHAWN RYNE
CARDIOMYOPATHY
• Cardiomyopathy is a group of diseases that affect the
heart muscle.
• Types of cardiomyopathy include hypertrophic
cardiomyopathy, dilated cardiomyopathy, restrictive
cardiomyopathy, arrhythmogenic right ventricular
dysplasia, and takotsubo cardiomyopathy (broken heart
syndrome).
DEFINITION
• Hypertrophic cardiomyopathy (HCM) is a condition in
which a portion of the heart becomes thickened without an
obvious cause.
• This results in the heart being less able to pump blood
effectively.
TYPES
• Acquired
• Asymmetric septal hypertrophy
• Familial hypertrophic cardiomyopathy
• Hypertrophic non obstructive cardiomyopathy
• Hypertrophic obstructive cardiomyopathy
• Idiopathic hypertrophic sub aortic stenosis (IHSS)
• Hypertrophic cardiomyopathy also can affect the heart's
mitral valve, causing blood to leak backward through the
valve.
• Sometimes, the thickened heart muscle doesn't block
blood flow out of the left ventricle.
• This is referred to as non-obstructive hypertrophic
cardiomyopathy.
• In both obstructive and non-obstructive HCM, the
thickened muscle makes the inside of the left ventricle
smaller, so it holds less blood.
• The walls of the ventricle may stiffen, and as a result, the
ventricle is less able to relax and fill with blood.
• This can raise blood pressure in the ventricles and the
blood vessels of the lungs. Changes also occur to the
cells in the damaged heart muscle, which may disrupt the
heart's electrical signals and lead to arrhythmias.
ETIOLOGY
• Familial hypertrophic cardiomyopathy is inherited as an
autosomal dominant trait and is attributed to mutations in
one of a number of genes that encode for the sarcomere
proteins.
• Long-term high blood pressure.
• Heart tissue damage from a heart attack.
• Chronic rapid heart rate.
• Heart valve problems.
• Metabolic disorders, such as obesity, thyroid disease or
diabetes.
PATHOPHYSIOLOGY
• In the most common phenotype, the anterior septum and
contiguous anterior free wall below the aortic valve are
markedly hypertrophied and thickened, with little or no
hypertrophy of the left ventricular (LV) posterior wall.
• Sometimes isolated apical hypertrophy occurs; however,
virtually any asymmetric pattern of left ventricular
hypertrophy can be observed, and in a small minority of
patients even symmetric hypertrophy has been noted.
• Hypertrophy results in a stiff, noncompliant chamber
(usually the left ventricle) that resists diastolic filling,
elevating end-diastolic pressure and thus increasing
pulmonary venous pressure. As resistance to filling
increases, cardiac output decreases, an effect worsened
by any outflow tract gradient present. Because
tachycardia allows less time for filling, symptoms tend to
appear mainly during exercise or tachyarrhythmias.
• Coronary blood flow may be impaired, causing angina
pectoris, syncope, or arrhythmias in the absence of
epicardial coronary artery disease (CAD).
• Flow may be impaired because capillary density relative
to myocyte size is inadequate (capillary/myocyte
imbalance) or lumen diameter of intramyocardial coronary
arteries is narrowed by intimal and medial hyperplasia and
hypertrophy.
SIGNS AND SYMPTOMS
• Could be asymptomatic
• Shortness of breath
• Exertional chest pain
• Heart palpitations
• Lightheadness, fainting, weakness/ syncope
• Sudden cardiac death
• Usually the onset is between 20-40 years.
RISK FACTORS FOR SUDDEN DEATH
• History of cardiac arrest and ventricular fibrillation
• Ventricular tachycardia
• Family of premature sudden death
• Unexplained syncope
• Abnormal blood pressure
DIFFERENTIAL DIAGNOSIS
• Aortic Stenosis
• Restrictive Cardiomyopathy