hypertrophic

cardiomyopathy 
 What is
cardiomyopathy ?

• Heterogeneous group of disease of

myocardium.
• In cardiomyopathy, the heart muscle
becomes enlarged, thick, or rigid. In rare
cases, the muscle tissue in the heart is
replaced with scar tissue.
• As cardiomyopathy worsens, the heart
becomes weaker. It's less able to pump blood
through the body and maintain a normal
electrical rhythm.
• This can lead to heart failure or
  complications, such as heart valve problems
.
 Cardiomyopathies

primary
Resulting from genetic Secondary
abnormalities or heart
Resulting from
muscle.
infections, metabolic and
• Dilated nutritional disease,
• Hypertrophic blood disease,tumors.
• restrictive
 Introduction

• 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
 Cause of HCM
Hypertrophic cardiomyopathy is usually caused
by abnormal genes (gene mutations) that cause
the heart muscle to grow abnormally thick.
People with hypertrophic cardiomyopathy also
have an abnormal arrangement of heart muscle
cells (myofiber disarray). This disarray can
contribute to arrhythmia in some people.
 Familial hypertrophic Cardiomyopathy
• Familial hypertrophic cardiomyopathy is
inherited as an autosomal dominant
• Mutations in genes that incode sarcomeric
proteins
• Approximately 40% of these mutations occur in
the β-myosin heavy chain gene on chromosome
14 q11.2-3,
• approximately 40% involve the cardiac myosin-
binding protein C gene.
• Some mutations could have more harmful
potential. For example, troponin T mutations
were originally associated with a 50% mortality
before the age of 40.
PATHOPHYSIOLOGY
The pathophysiology of HCM involves 4 interrelated processes:
• Thickening of septum may cause LV outflow obstruction
and abnormal motion of mitral valve.
• Abnormal motion can cause mitral regurgitation.
• Diastolic disfunction
• Myocardial ischemia
Hypertrophic CARDIOMYOPATHY
PATHOLOGIC FINDINGS
 Signs and Symptoms
• Shortness of breath, especially during exercise
• Chest pain, especially during exercise
• Fainting, especially during or just after exercise or exertion
• Sensation of rapid, fluttering or pounding heartbeats
(palpitations)
• Heart murmur, which a doctor might detect while listening to
your heart