Non-Ischaemic Myocardial Disease

Question 1

What is a cardiomyopathy

Answer 1

Any disease of the cardiac muscle that is not ischaemic, hypertensive

Question 2

What changes may cardiomyopathy cause?

Answer 2

Changes in size of chamber walls

Question 3

What are the four classifications of cardiomyopathy?

Answer 3

Dilated
Hypertrophic
Restrictive
Arrhythmogenic right ventricular dysplasia

Question 4

Describe dilated cardiomyopathy?

Answer 4

Weak and flabby heart

Bigger than it should be may only be by 50g though

Question 5

What are the histological features of dilated cardiomyopathy?

Answer 5

none

Question 6

What are the causes of dilated cardiomyopathy?

Answer 6

50% genetic
toxins
-alcohol
-doxorubicin (chemo)
cardiac infection
pregnancy

Question 7

What are the clinical features of DCM?

Answer 7

General heart failure

Question 8

Describe hypertrophic cardiomyopathy?

Answer 8

Big solid heart
Strong contraction
Heart doesn’t relax as it should

Question 9

What does hypertrophic cardiomyopathy cause?

Answer 9

Outflow obstruction

Sudden death

Question 10

What are the causes of HCM?

Answer 10

Genetic
Actin or myosin mutated genes
beta myosin heavy chain

Question 11

What is the appearance of HCM?

Answer 11

RV and RA look normal
LA may be dilated
LV will be extraordinarily thick- normally 15mm, may be 4cm
Coronary Arteies are not increased and therefore there must be areas of relative hypoxia or ischaemic- possibly angina

Question 12

What is the histology of HCM?

Answer 12

Myofibres will be disorganised (swirls-myofibre disarray) as opposed to linear

Question 13

Describe restrictive cardiomyopathy?

Answer 13

Lack of compliance
Stiff heart
Doesn’t fill well- diastolic dysfunction
Can’t generate a sufficient stroke volume to generate BP
Bi-atrial dilatation as a result of back pressure

Question 14

What does RCM look like histologically?

Answer 14

Normal

Question 15

What are the causes of RCM?

Answer 15

>Deposition of something in the myocardium 
>metabolic by-products (iron)
>amyloidosis
>sarcoidosis
>tumour
>fibrosis

Question 16

Describe amyloidosis

Answer 16

o abnormal deposition of an abnormal protein throughout the body
o lots of different types of protein- lots of types of amyloid
o tendency to form beta-pleated sheets
o body cannot get rid of them

Question 17

What kind of amyloidosis is isolated to the heart?

Answer 17

Senile cardiac amyloidosis

Question 18

What kind of amyloidosis may be isolated to the atrium?

Answer 18

Abnormal atrial natriuretic protein

Question 19

What is the appearance of amyloidosis on histology?

Answer 19

o waxy pink material
o eosinophilic
o exhibits apple green birefringence
o stains positively for Congo red

Question 20

What does amyloid generally resemble?

Answer 20

RCM

Question 21

What is arrhythmogenic right ventricular dysplasia?

Answer 21

> Right ventricle becomes largely replaced by fat

>Big and floppy

Question 22

What does ARVD look like histologically?

Answer 22

only a tiny bit of myocardium and muscle is replaced by adipocytes

Question 23

What is the cause of ARVD?

Answer 23

Genetic

Question 24

What is myocarditis?

Answer 24

Inflammation of the heart

Question 25

What are the types of myocarditis?

Answer 25

Infectious vs non infectious | Bacterial, viral, fungal, protozoal and helminthic

Question 26

What is the most common type of myocarditis?

Answer 26

Infectious

Question 27

What are the causes of infectious myocarditis?

Answer 27

- Coxsakie A and B - ECHO virus - chaga's disease- trypanosomiasis in S. America - borrelia burgdorferi- Lyme's disease - HIV

Question 28

What does the myocardium look like in infectious myocarditis?

Answer 28

Thick and beefy

Question 29

What might you see on biopsy of infectious myocarditis?

Answer 29

Inflammatory infiltrate

Question 30

What are the causes of non-infectious myocarditis?

Answer 30

>Immune mediated hypersensitivity disorders >hypersensitivity to infection >hypersensitivity to drugs >SLE

Question 31

Describe hypersensitivity to infection in myocarditis?

Answer 31

rheumatic fever after strep sore throat o classic mitral stenosis with thickening and fusion of valve leaflets o short, thick chordae tendinae o myocardium is also patchily inflamed o fibrotic scarring will effect heart valves and give you mitral stenosis

Question 32

What will you see histologically in myocarditis caused by hypersensitivity to infection?

Answer 32

o histologically- aschoff bodies, central destructive region which peppers the heart valves

Question 33

What is pericarditis?

Answer 33

Inflammation of the pericardial layers | Outer layers of the heart become inflamed

Question 34

What are the causes of pericarditis?

Answer 34

``` Infectin Immune related Idiopathic Uraemic Post MI SLE ```

Question 35

What is post MI pericarditis?

Answer 35

Dressler's syndrome - abnormal response to dead cells - many weeks post MI

Question 36

Which virus produces serious effusion in pericarditis?

Answer 36

ECHO

Question 37

What is the common cause of bacterial pericarditis?

Answer 37

extension from elsewhere, pneumonias, produce purulent effusions

Question 38

Who gets fungal pericarditis?

Answer 38

immunosuppressed pts, post-transplant (bone marrow for example), produce purulent effusions

Question 39

What are the complications of pericarditis?

Answer 39

``` >pericardial effusion >tamponade >constrictive pericarditis >cardiac failure >death ```

Question 40

Describe constrictive pericarditis?

Answer 40

- fibrotic response to inflammation on surface of the heart - wherever there is fibrosis there is collagen, which contracts and cause a contracture, strangling the heart from the inside

Question 41

What is endocarditis?

Answer 41

inflammation of the inside of the heart lining generally referring to the inflammation of the valves

Question 42

Where does infective endocarditis occur?

Answer 42

can occur at normal valves

Question 43

What does infective endocarditis usually require?

Answer 43

A very virulent organism

Question 44

What are the most common causes of infective endocarditis now?

Answer 44

prosthetic valves, congenital defects, bicuspid valves, MV prolapse, calcific disease in the elderly

Question 45

What are the HACEK organisms?

Answer 45

Organisms of endocarditis - haemophilus - aggregatibacter - cardiobacterium - eikenella corrodens - kingella king

Question 46

What is the pathology of IV drug users?

Answer 46

Right sided valves

Question 47

Which organism is common in prosthetic valves?

Answer 47

S. epidermidis

Question 48

What is a vegetation

Answer 48

aggregates of organisms on heart valves

Question 49

What are some common signs of endocarditis?

Answer 49

- oslers nodes - janeway lesions - roth spots - splinter haemorrhages - septicaemia

Question 50

What is non-infectious endocarditis?

Answer 50

Marantic

Question 51

What is non-bacterial thrombotic endocarditis?

Answer 51

Smaller

Question 52

What is non-bacterial thrombotic endocarditis associated with?

Answer 52

cancers | o mucinous adenocarcinomas

Question 53

What is found on the valves in non-bacterial thrombotic endocarditis ?

Answer 53

Small and multiple vegetation | non-invasive and don't destroy valves

Question 54

When is non-bacterial thrombotic endocarditis common?

Answer 54

In hyper-coagulable states

Question 55

What kind of endocarditis occurs in lupus?

Answer 55

Libman-sacks endocarditis

Question 56

What are the symptoms of Libman-sacks endocarditis?

Answer 56

Often asymptomatic

Question 57

What kind of emboli are found in Libman-sacks endocarditis?

Answer 57

Small sterile emboli

Question 58

What is carcinoid heart disease?

Answer 58

Neuroendocrine cancer | can see them in any mucosa- common in GI and lung

Question 59

Where do carcinoid tumours metastasise to?

Answer 59

The liver

Question 60

What kind of heart disease does carcinoid heart disease produce?

Answer 60

Right sided cardiac valve disease | Tricuspid and pulmonary insufficiency

Question 61

What is the commonest heart tumour?

Answer 61

Atrial myxoma is the commonest primary

Question 62

Where is atrial myxoma found?

Answer 62

sits just above the mitral valve

Question 63

Is atrial myxoma benign?

Answer 63

Yes but can produce clinical pathology that is just as bad as malignancy

Question 64

What is a side effect of atrial myxoma?

Answer 64

Myxoid tumour emboli May develop endocarditis Systemic fever and malaise, IL-6 Potential secondary

Question 65

Where are atrial myxoma found?

Answer 65

90% in atria, usually left