Cardiomyopathies

Question 1

Cardiomegaly

Answer 1

Big heart

Question 2

Cardiac dilation

Answer 2

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

Question 3

What is the white stuff that is shown when the heart is cut in vertical section

Answer 3

Subendoacardial fibrosis

This is dilated cardiopathy

Question 4

Answer 4

2 D’s, classic of dilated cardioopathy

Question 5

What are the pathological changes that take place in dilated cardiopathy

Answer 5

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

Question 6

Answer 6

There is more stroma than muscles

Question 7

Secondary dilated cardiopathies, why do they happen

Answer 7

1. Toxic cardiomyopathy, like in chemotherapy
2. Cardiomyopathy of pregnancy, happens in the last trimester, it has autorecovery half of the time
3. Viral cardiomyopathy, myocarditis

Question 8

What are the clinical features of dilated cardiomyopathy

Answer 8

1. Asymptomatic
2. Progressive exercise intolerance
3. CHF

There is no other intervention other than a cardiac transplant

Question 9

Hypertrphic cardiomyopathy

Answer 9

1. Usually involves left ventricle but it can also involve both of the ventricles, the median septum may also thicken
2. There is assymetry hypertrophy, also called obstructuve cardiomyopathy
3. There are genetic factos associated with this disease

Question 10

Answer 10

Septum is thicker, this is hypertrophic cardiomyopathy

Question 11

Answer 11

Myofiber disarray, this happens in hypertrophic cardiomyopathy

Question 12

Contrast hypertrophic and dilated cardiomyopathy, from what we have learned so far

Answer 12

In dilated there is global enlargement of the heart, whereas in hypertrophic usually the ventricles are involved

Question 13

What are the clinical features of hypertrophic cardiomyopathies

Answer 13

1. Asymptomatic
2. Associated with sudden death while playing sports or exercising
3. CHF like symptoms

Question 14

Restrictive cardiomyopathy

Answer 14

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

Question 15

Pathology of restricitve cardiomyopathies

Answer 15

Fibrosis happens which can dislodge from the heat and occlude a blood vessel. In the pathology we see

1. Grayish white thickened endocardium
2. Mural thrombu
3. Fibrotic endocardium

Question 16

Answer 16

Restrictive cardiomyopathy

Question 17

What are the group of diseases that can predispose someone to restrictive cardiomyopathies

Answer 17

1. Amyloidosis
2. Saracoidosis
3. Storage diseases
4. Idiopathic

Question 18

Amyloidosis

Answer 18

1. Related to mutiple myeloma
2. Pathology involves cardiomegaly and amyloid infilterate
3. Clinically can be seen as right sided heart failure and arrythmias

4.

Question 19

Answer 19

Cardiac amyloidosis

Question 20

What disease is common between dilated and restrictive cardiomyopathies

Answer 20

Sarcoidosis, it is associated with granulomas

Question 21

Storage disease

Answer 21

Glycogen storage diseases etc etc

Hemachromatosis

Question 22

Arrythmogenic cardiomyopathies

Answer 22

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

Question 23

Answer 23

Arrythmagenic cardiomyopathy

Question 24

Summary

Answer 24