Cardiovascular Pathology

Question 1

What is cardiomyopathy?

Answer 1

Changes in the size of the heart and changes in the thickness of chambers of the heart

Question 2

What are the five main types of cardiomyopathy?

Answer 2

Dilated
Restrictive
Hypertrophic
Arrhythmogenic right ventricular dysplasia
Amyyloidosis

Question 3

What is dilated cardiomyopathy

Answer 3

Heart 2-3x normal (450g)
Hypertrophic changes but soft - flabby and floppy
Down microscope - features non-specific, some hypertrophy (not very helpful in making diagnosis)

Question 4

Causes of dilated cardiomyopathy

Answer 4

Genetics - about 50% of all
-AD,AR, X-linked, mitrochondrial
-These mutations are in the genes that encode for heart muscle proteins

Toxins related e.g alcohol

Doxorubicin - chemotherapy agents (must asses heart before starting chemotherapy)

Childbirth - late in pregnancy or post partum

Cardiac Infections e.g. Myocarditis

Question 5

Clinical features of dilated cardiomyopathy

Answer 5

General size/‘picture’ of the heart
SOB, poor exercise tolerance etc
Low ejection fraction (low Cardiac Output)

Question 6

What is hypertrophic cardiomyopathy

Answer 6

Big solid hearts
Hypertrophic and string contraction (but not like DCM)
Diastolic dysfunction (problem with filling)- systolic contraction is fine
Eventually outflow obstruction

Assoc. with sudden death - athletes

Question 7

What is the causes of hypertrophic myopathy

Answer 7

Genetics (mutations of lots of different genes)

Question 8

What is restrictive cardiomyopathy?

Answer 8

A lot of resistance/lack of compliance of heart - stiff
Diastolic dysfunction - doesnt fill well
Can look normal (unlike DCM or HOCM)
Biatrial dilatation as a result of back pressure from ventricles (no give in ventricles)

Question 9

What is the causes of restrictive cardiomyopathy?

Answer 9

Something in heart muscle wall stopping it pumping well e.g metabolic byproducts like iron
Amyloid
Sarcoid - multi system granulomatous disorder
Tumours (very rare)
Radiation fibrosis (very rare)

Question 10

What is Arrhythmogenic right ventricular dysplasia?

Answer 10

A genetic disease - autosomal dominant but with a low penetrance (may inherit disease but dont always get)
It is where the right ventricle muscle is largely replaced by fat = big and floppy
Trouble with this is that the right ventricle always looks a bit fatty

Very difficult to diagnose

Question 11

How can Arrhythmogenic right ventricular dysplasia present itself?

Answer 11

Underlying Arrhythmias - so patients present with syncope and funny turns
Sudden death may occur

Question 12

What is myocarditis?

Answer 12

Inflammation of the heart - usually infectious

Question 13

What is the most common cause of infectious myocarditis

Answer 13

Infection: viral, bacterial, fungal, protozoal and helminthic

Most is viral - coxsackie A and B, ECHO virus most common
There is other ones as well - chagas disease, HIV and lyme’s disease

(Lymes disease can cause sudden death as it block AV node)

Question 14

What is the most common cause of non-infectious myocarditis

Answer 14

Usually immune mediated hypersensitivity reactions e.g to drugs
Hypersensitivity to infection - poststreptococcal and rheumatic fever
Systemic lupus erythematosus (SLE) - hypersensitivity
Aschoff bodies (microscopic - another form of rheumatic granuloma) - be aware of for paediatrics

Question 15

What is amyloid?

Answer 15

Generally resembles a type of restrictive cardiomyopathy’s
Multisystem disease (probs not a single system that will cover it well)
Abnormal deposition of any protein - abnormal proteins deposited throughout body (lots of diff types as lots of diff proteins)
Tendency to form beta pleated sheets - this means its more difficult for body to get rid of so debri builds up and impaire function of where these proteins are deposited

Is a cause of arrhythmogenic sudden death
At autopsy - often effects conduction pathway of heart

Question 16

What are the different types of amyloid?

Answer 16

AA - related to chronic diseases e.g chronic autoimmune diseases like rheymatoid arthritis - abnormal antibodies
AL - light chains, abnormal immunoglobin - in the presence of haematological neoplasia and cancer
Haemodialysis associated amyloidosis - will be given beta 2 microglobulin deposition (dont look for other diseases in this situation)
Familial forms:
-Diabetes
-Alzheimer’s

Question 17

What does amyloid look like/how to identify?

Answer 17

Waxy pink material (clue will get in exam)
Stains positively for “congo red”
Exhibits apple green birefringence

Question 18

Amyloid can be isolated to the heart. What is it called when amyloid is localised in the atrium?

Answer 18

Abnormal atrial natriuretic protein

Question 19

What is the desposition of normal transthyretin called?

Answer 19

Senile cardiac amyloidosis - ‘normal’ consequence of aging but also can be inherited

Question 20

What is pericarditis?

Answer 20

Inflamation of the pericardium

Question 21

What are the causes of pericarditis?

Answer 21

Main cases:
Infection:
-ECHO virus (coxaci A and B), extension from elsewhere, generally serous (discoloured but clear fluid), suppurative (bacterial - white pus, cheesy looking) -TB

Immune mediated
e.g. post MI - dresslers syndrome, uraemia - high serum urea levels occur and renal failure, connective tissue diseases (e.g. rheumatoid, lupus)

Weird causes

Question 22

What is Dressler’s syndrome?

Answer 22

Many weeks (6) after MI - late complications of MIs
Assumed to be immune mediated - damaged heart muscles release preciously un-encountered material that stimulates an immune response

Question 23

What is endocarditis?

Answer 23

Inflammation of the inside lining of the heart - generally refers to the valves
Mostly caused by infection - specifically bacteria

Question 24

How does infectious endocarditis work?

Answer 24

Colonies of bugs on valves (vegetations). They are loose on the valves and with turbulent blood flow can come off and spread throughout body - emboli

Question 25

What bacteria is the most common cause of infectious endocarditis?

Answer 25

Strep Viridians HACEK - Haemophilus, Actinobacillus, Cardiobacteria, Eikenella, Kingella Staph aureus - IV drug users, right side of heart Staph epidermidis - prosthetic valves

Question 26

What are the common causes of infectious endocarditis?

Answer 26

Rheumatic valves, prosthetic valves (more likely to have vegetation’s forming on them), congenital defects, bicuspid valves, MV prolapse, calcification disease Lupus Non-bacterial - marantic Carcinoid disease Can also see on normal valves not that’s not common

Question 27

What are the clinical features of infectious endocarditis?

Answer 27

Olsers nodes Janeway lesions Roth spots Splinter haemorrhages Systemic septic emboli - brain, kidney, lungs etc

Question 28

What does libman-sacks endocarditis?

Answer 28

Libman - sacks is what you see in lupus Small sterile emboli Vegitations on the underside and on top of the valves or on chords The changes range - small asymptomatic deposits or significant valvulitis

Question 29

What is non-bacterial thrombotic endocarditis (NBTE)?

Answer 29

Can cause emboli disease but tends not to destroy valves therefore doesn’t destroy heart as much - Non-invasive Can form non-bacterial thrombosis on valves - sterile vegitations (marantic endocarditis) this is normally associated with malignancy in hypercoagulability states (e.g. frequently mucinous adenocarcinomas)

Question 30

What is carcinoid heart disease?

Answer 30

Carcinoid tumours are neoplasms of neuroendocrine cells They are seen in any mucosa - most commonly in GI tract and lung Associated with release of hormone (hence neuroendocrine) Carcinoid syndrome occurs only when the heart is involved becuase there’s metastases of liver - cause of carcinoid heart disease (metastases of liver)

Question 31

What is the clinical presentation of carcinoid syndrome? And why?

Answer 31

Liver must be metastasised so releases 5HIAA, serotonin, histamine, bradykinin etc checked in urine This causes: Flushing of skin Nausea, vomiting and diarrhea Right sided cardiac valve disease which causes tricuspid and pulmonary insufficiency (mechanism unknown)

Question 32

What are the cardiac tumours?

Answer 32

Don’t really get cardiac tumours Benign tumour of myocardium - rhabdomyomas Tumours of blood vessels - angiomas, angiosarcomas (incredibly rare) Myxoma - most common but still very rare

Question 33

What are (atrial) myxoma tumours?

Answer 33

Most common but still very rare Almost always in atria (90%) and usually in the left atrium This can cause a ball/valve obstruction and myxoid embolism

Question 34

What is carney’s syndrome? (This is nitty gritty so dw too much but will come across - possibly wait till everything else is more solidified)

Answer 34

Associated with atrial myxomas Multiple myxomatosis on skin Pigmented skin lesions Neurofibromas Rare calcifying Sertoli cell tumours of testicle Pancreatic and thyroid tumours PRKAR1A gene