lec 2 Cardiomyopathy Flashcards
Cardiac dysfunction caused by
CARDIOMYOPATHIES, less common
Secondary Myocardial Dysfunction (Mimicking Cardiomyopathy) , more common
Cardiomyopathy Types
1.Dilated cardiomyopathy (DCM) (including
arrhythmogenic right ventricular cardiomyopathy)
2.Hypertrophic cardiomyopathy (HCM)
3.Restrictive cardiomyopathy
Hypertrophic dysfunction in
Diastolic dysfunction
Hypertrophic cardiomyopathy يعني
thickend left ventricular wall
myocardial hypertrophy,
defective diastolic filling,
and— in one third of cases—ventricular outflow obstruction
most common type of Cardiomyopathy
dilated Cardiomyopathy
Dilated cardiomyopathy يعني
- enlargement of all cardiac chamber→ cardiac dilation and poor contractility
- Flabby
- wall thinning.
Restrictive cardiomyopathy يعني
rigid ventricular wall
risk in sudden death in young athletes
Hypertrophic dysfunction
DCM causes
Genetic causes in 20% to 50% of DCM cases mainly autosomal dominant (AD) inheritance→ Cytoskeletal proteins or proteins that link the sarcomere to
the cytoskeleton
the most common manifestation in iron
overload patients
DCM
DCM diagnostic age
between 20 and 50 years of age
DCM treatment
Cardiac transplantation
Mural thrombi are often present and may be a source of thromboemboli in
DCM
DCM Manifests with
progressive CHF, including dyspnea, easy fatigability, and poor exertional capacity.
• One half of patients die within 2 years (cardiac failure or Arrhythmia)
The heart in DCM characteristically is
enlarged
The characteristic histologic abnormalities in DCM are
iron overload
↑ intramyocardial hemosiderin.
myocytes exhibit hypertrophy with enlarged nuclei
fibrosis
cardiomyopathy common
DCM
cardiomyopathy common
DCM > HCM > RCM
HCM Genetic causes
Missense mutation → autosomal dominant (AD) inheritance → proteins that form the contractile apparatus in particular βmyosin heavy chain
the different between HCM and DCM
1. IN HCM The heart is thick-walled IN DCM The heart is thin walled 2.The heart in DCM is Flabby The heart in HCM is not flabby 3.The heart in DCM is poorly contractile The heart in HCM is hypercontractile
IN HCM the heart is
heavy
myocardial hypertrophy without ventricular dilation
asymmetric septal hypertrophy)
asymmetric septal hypertrophy)
Disproportionate thickening of the ventricular septum relative to the left ventricle free wall
HCM….. MORPHOLOGY
banana-shaped” ventricular lumen
HCM age
post pubertal growth spurt…but can occur at any age
Restrictive Cardiomyopathy (RCM) Causes
– Primary, Idiopathic (unknown cause) – Secondary to systemic diseases: • radiation fibrosis, • amyloidosis, • sarcoidosis, • inborn errors of metabolism.
RCM Characterized by
a primary decrease in ventricular
compliance, resulting in impaired ventricular filling during diastole
RCM شو وضع القلب
ventricles are normal size or only slightly enlarged
vantricular cavities in RCM are
not dilated,
myocardium in RCM IS
firm
Both atria in RCM are
dilated as a consequence of restricted ventricular filling and pressure overloads
Microscopic examination reveals in RCM
interstitial fibrosis.
Endomyocardial biopsy often can reveal in RCM
Amyloidosis, sarcoidosis
