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Transcript of CardioMyoPathies
Chrarcterized by primary decrease in ventricular compliance, resulting in impaired ventricular filling during diastole (the wall is stiffer). 1/primary: idiopathic
2/secondary (associated with systemic diseases )
Common: Amyloidosis, Endomyocardial fibrosis
Uncommon: Sarcoidosis, Loeffler- endomyocarditis
Rare: Scleroderma ,Storage Arrhythmogenic right ventricular cardiomyopathy
- ( Rt HF + ventricular tachy. )> sudden death. Because of:
The ventricles are rigid ,less elasticity and noncompliant
So, the ventricles will be filled rapidly before the whole blood leave the atrial cavities.
The systemic blood flow will be affected because of
cardiac input ≠ cardiac output
because cardiac output is decreased in systemic circulation so organs can suffer from less blood supply . Objectives
1. Define cardiomyopathy
2. Classify cardiomyopathy
3. Mention etiological factors of each type of cardiomyopathy
4. Briefly describe the morphology of each type of cardiomyopathy CardioMyoPathies Classification 1/ Dilated CardioMyoPathies
2/ HyperTrophic CardioMyoPathies
3/ Restrictive CardioMyoPathies Thank You For Listening. Morphlogy Of RCM Morphology Of HCM Morphology Of DCM Morphology Cardiomegaly:
LV/bi ventricular dilatation:
The heart in DCM is characteristically enlarged (two to three times its normal weight) and flabby, with dilation of all chambers.
•Mural thrombi are common and may be a source of thromboemboli
•most myocytes are hypertrophied with enlarged nuclei, but many are attenuated, stretched, and irregular.
•There is variable interstitial and endocardial fibrosis: scattered scars are also often present Gross picture:
•- massive myocardial hypertrophy without ventricular dilation
•- disproportionate thickening of the ventricular septum relative to the left ventricle free wall
•- On longitudinal sectioning, the ventricular cavity loses its usual round-to-ovoid shape and is compressed into a "banana-like" configuration
•- severe myocyte hypertrophy
•- myocyte (and myofiber) disarray
•- interstitial and replacement fibrosis HyperTrophic Cont. Clinical Features Mechanism Not clearly understood.
Thought to be a compensatory change in response to impaired contractility.
Ineffective myocyte contraction stimulate the release of abundant growtha factors →
intense compensatory hypertrophy (Myofiber Disarray) &
fibroblast proliferation(Interstitial fibrosis). Unique, involving right-sided heart failure and rhythm disturbances. Summery HyperTrophic Known as Pathogenesis Causes Idiopathic hypertrophic sub aortic stenosis.
Heart is thick-walled, heavy, hyper contracting Vs. flabby hypcontracting of DCM. - Misssense point mutations in one of the genes encoding sarcomeric proteins
- Autosomal dominant.
- 100 mutations in at least 12 sarcomeric genes. Characteristics Myocardial hypertrophy, Abnormal diastolic filling, 1/3 of cases: ventricular outflow obstruction. The 4th CardioMyoPathy Pathogenesis Problem in the ventricle (during diastole) especially in the myocardium( firm and non compliant less elasticity)
The ventricle contractile function (during systole ) usually unaffected.
Biatrial dilatation is common. 1- Endomyocardial fibrosis
Disease of children and young adults in Africa and other tropical areas; it is characterized by dense fibrosis of the ventricular endocardium and subendocardium extending from the apex up to the tricuspid and mitral valves, the fibrous tissue from organization of mural thrombi.
Causes endocardial fibrosis, typically with large mural thrombi. there is often an associated peripheral hypereosinophilia; the circulating eosinophils are abnormal, and many are degranulated.
Release of the eosinophil granule contents, especially major basic protein, is speculated to initiate endocardial damage, with subsequent endomyocardial necrosis followed by scarring of the necrotic area. 3/endocardial fibroelastoses
Uncommon .. First 2 years .. decompensation and death. Heart Diseases resulting from a 1ry abnormality in myocardium.
# ischemic CMP " congestive HF caused by CAD ". Common Symptoms Dyspnea (breathlessness) and peripheral edema (swelling of the legs).
People with cardiomyopathy are often at risk of dangerous forms of irregular heart beat and sudden cardiac death.
The most common form of cardiomyopathy is dilated cardiomyopathy. Genetic Associated
1/ β Myosin heavy chain.
2/ Myosin binding protein C
3/ Troponin T Diseases Associated
Amyloidosis, Fabry's Disease,
Hypertensive Heart Disease with age related sub aortic septal hypertrophy. Massively Hypertrophic left ventricle → reduced stroke volume ( due to impaired diastolic filling)
25% Dynamic obstruction to the left ventricular out flow by Ant. leaflet of mitral valve.
Reduced cardiac output.
Secondary increase venous pressure.
High systolic ejection murmur
Myocardial ischemia (due to massive hypertrophy, high LV pressure & compromised intramural arteries )
Mural Thrombus, Infective endocartitis,
Arrhythmia (Atrial fibrillation)
SCD Morphology RV wall severely thinned.
result of Myocyte replacement by massive fatty infiltration & lesser amount of fibrosis Causes Mutations in Ch. 14 (desmosomal junction proteins)
Autosomal dominant. - Congestive cardiomyopathy
-Progressive cardiac hypertrophy , dilation , contractile dysfunction. (Systolic)
Due to heart with dilated chambers and thin walls not being able to contract forcefully enough to supply body with an adequate amount of blood leading to >> CHF . Gross picture:
•- The ventricles are of approximately normal size or slightly enlarged, the cavities are not dilated, and the myocardium is firm.
•- Biatrial dilation is commonly observed.
•- interstitial fibrosis, varying from minimal and patchy to extensive and diffuse
•- disease-specific features can be seen on endomyocardial biopsy (e.g., amyloid, iron overload, sarcoid granulomas). Myocardial diseases Secondarily :
IHD ,HPT,VHD Primary:
Intrinsic to myocardium Disease(fabry,gaucher,hurlur,glycogen storage)
hemochromatosis ,RaSclerodermadiation ,Metastatic tumor
3/Genetic factors: (Less clearly defined in restrictive cardiomyopathy) Cuases Cont. Supervisor; Dr. Naseemah Etiology 20-60 yrs , slowly progressive CHF , EF <25% , death within 2yrs ( CHF or Arrhythmia ) , embli.
Same as CHF :