Cardiomyopathies Flashcards
Cardiomegaly
Big heart
Cardiac dilation
There is hypertrophy of all 4 chambers of the heart which causes the heart to become bigger than normal - it is called the globoid enlargement
Heart will have a lot of fibrous tissue so the heart is going to be very soft and pliable
What is the white stuff that is shown when the heart is cut in vertical section
Subendoacardial fibrosis
This is dilated cardiopathy
2 D’s, classic of dilated cardioopathy
What are the pathological changes that take place in dilated cardiopathy
There is hypertrophy and at the same time atrophy in some regions of the cardiac myocytes.
Also you would notice that there is fibrosis of the heart.
There is increased mitochondria and loss of sarcomeres
There is more stroma than muscles
Secondary dilated cardiopathies, why do they happen
- Toxic cardiomyopathy, like in chemotherapy
- Cardiomyopathy of pregnancy, happens in the last trimester, it has autorecovery half of the time
- Viral cardiomyopathy, myocarditis
What are the clinical features of dilated cardiomyopathy
- Asymptomatic
- Progressive exercise intolerance
- CHF
There is no other intervention other than a cardiac transplant
Hypertrphic cardiomyopathy
- Usually involves left ventricle but it can also involve both of the ventricles, the median septum may also thicken
- There is assymetry hypertrophy, also called obstructuve cardiomyopathy
- There are genetic factos associated with this disease
Septum is thicker, this is hypertrophic cardiomyopathy
Myofiber disarray, this happens in hypertrophic cardiomyopathy
Contrast hypertrophic and dilated cardiomyopathy, from what we have learned so far
In dilated there is global enlargement of the heart, whereas in hypertrophic usually the ventricles are involved
What are the clinical features of hypertrophic cardiomyopathies
- Asymptomatic
- Associated with sudden death while playing sports or exercising
- CHF like symptoms
Restrictive cardiomyopathy
These are a group of diseases where the diastolic filling of the heart is impaired such that the heart doesnt fill with sufficient quantities of blood. The contractile function of the heart remains normal
There are 2 ends of the spectrum
Endomyocardial Fibrosis- less aggressive one, happens in young children, leads to CHF and death
Loeffler Endocarditis - happens in men in their 5th decade of their life, more aggressive, CHF and death. Assocaited with hypereosinophilia and myeloperoxidase disorder
Endocardial fibroelastosis - happens in the first 2 years of life, here the pump function of the heart is affected as the LV is the one which is most affected
Pathology of restricitve cardiomyopathies
Fibrosis happens which can dislodge from the heat and occlude a blood vessel. In the pathology we see
- Grayish white thickened endocardium
- Mural thrombu
- Fibrotic endocardium
Restrictive cardiomyopathy
What are the group of diseases that can predispose someone to restrictive cardiomyopathies
- Amyloidosis
- Saracoidosis
- Storage diseases
- Idiopathic
Amyloidosis
- Related to mutiple myeloma
- Pathology involves cardiomegaly and amyloid infilterate
- Clinically can be seen as right sided heart failure and arrythmias
4.
Cardiac amyloidosis
What disease is common between dilated and restrictive cardiomyopathies
Sarcoidosis, it is associated with granulomas
Storage disease
Glycogen storage diseases etc etc
Hemachromatosis
Arrythmogenic cardiomyopathies
This diseases is characterized by gradual deposition of adipose and fibroadipose which replace the cardiomyocytes in the ventricles
Also called right ventrucle rythmagenic dysplasia
Bu histology we will see a ton of fact
Arrythmagenic cardiomyopathy
Summary
