Cardio-Vascular pathology

Question 1

Heart Failure Definition

Answer 1

Inability of heart to pump blood at rate commensurate with the requirements of the metabolising tissues

Question 2

HF usually develops…

Answer 2

Slowly on background of cardiac hypertrophy

BUT may be acute e.g. MI, acute valve dysfunction

Question 3

HF Systolic dysfunction causes

Answer 3

Ischaemic heart disease
Pressure or volume overload such as systemic hypertension or AS
Cardiomyopathy

Question 4

HF Diastolic dysfunction causes

Answer 4

Inability to relax/fill
Amyloid
Myocardial fibrosis
Constrictive pericarditis

Question 5

HF problems forwards + backwards

Answer 5

Decreased output

Damming of blood in venous system

Question 6

HF Compensation mechanisms

Answer 6

Frank-Starling law: stretching leads to increased contraction •hypertrophy +/-chamber dilatation
•activation of neurohormonal systems including noradrenaline, RAS and atrial natriuretic peptide

Question 7

HF Consequences

Answer 7

Heart size/weight increase
•hypertrophy not hyperplasia (sudden death)
•pressure overload -concentric hypertrophy
•volume overload -cavitary dilatation maybe without thickening
•hypertrophy -capillary decrease -increase in fibrous tissue -increased metabolic needs -vicious circle ——> failure

Question 8

Left sided HF- main features

Answer 8

Decreased peripheral pressure + damming of blood in pulmonary circulation
brain + kidneys affected if severe (hypoxia)

Question 9

Left sided HF symptoms

Answer 9

Lungs congested
Oedema
Accumulation of haemosiderin laden macrophages in alveoli- leads to dyspnoea, orthopnoea, PND

Question 10

Right sided HF

Answer 10

Usually secondary to LVF

Primary association with severe pulmonary HT

Question 11

Right sided HF autopsy

Answer 11

Liver congested- “nutmeg” appearance, centrilobular necrosis + fibrosis

Question 12

Right sided HF signs

Answer 12

Portal vein pressure increase- splenomegaly, ascites

Kidneys + brain hypoxia, peripheral oedema

Question 13

Hypertension

Answer 13

Elevated BP
Systolic >140, Diastolic >90
Important RF for IHD, CVD (cerebro vascular disease), aortic dissection, cardiac failure, renal failure
Primary/essential (95%)
Secondary
"benign" or "malignant/accelerated" (5%)

Question 14

Biventricular failure

Answer 14

Both Right and Left side features

Question 15

Secondary Hypertension causes

Answer 15

Renal
Neurological
Cardiovascular
Endocrine

Question 16

2dary hypertension- renal

Answer 16

Chronic renal disease
Glomerulonephritis
Polycystic kidney disease
Renal artery stenosis
Vasculitis
Renin tumour

Question 17

2dary hypertension- neurological

Answer 17

Stress inducing surgery
Psychogenic
Raised intracranial pressure

Question 18

2dary hypertension- cardiovascular

Answer 18

Coarctation of aorta
Systemic vasculitis
Increased intravascular vol. or CO

Question 19

2dary hypertension- endocrine

Answer 19

Adrenocortical hyperfunction (Cushings/Conns)
Exogenous hormones
Phaeochromocytoma
Acromegaly
Thyroid disease (hyper or hypo)
Pregnancy

Question 20

BP equation

Answer 20

BP = Cardiac Output x Peripheral Resistance

Question 21

Hypertension pathological features

Answer 21

LVH
Vessels- atheroma, dissection, cerebrovascular haemorrhage, degenerative changes such as fibrointimal thickening
Small vessels- hyaline arteriolosclerosis esp. kidneys, hyperplastic arteriosclerosis (onion skinning), fibrinoid necrosis in vessel wall

Question 22

Cor pulmonale

Answer 22

Heart disease consequent to lung disease

Question 23

Pulmonary HT

Answer 23

High BP in pulmonary circulation

Question 24

Pulmonary HT/Cor pulmonale causes

Answer 24

Disease of lung parenchyma e.g. COPD, cystic fibrosis, diffuse interstitial fibrosis
Diseases of pulmonary vessels e.g. recurrent PEs, primary PH, severe vasculitis
Disorders affecting chest movement e.g. kyphoscoliosis, neuromuscular disease
Disorders causing arterial constriction e.g. hypoxaemia, chronic altitude sickness

Question 25

Cor pulmonale

Answer 25

Acute or chronic (acute with passive PE, normally chronic) See RV hypertrophy, RA hypertrophy + dilatation of both chambers Tricuspid regurg Clinical features as of RVF plus primary disease

Question 26

Thickened R ventricle criteria

Answer 26

Normal <0.5cm

Question 27

Vasculitis

Answer 27

Inflammation of vessel walls Infectious or non-infectious Non-infective are immune complex, ANCA mediated, direct antibody mediated, cell mediated, paraneoplastic or idiopathic

Question 28

Vasculitis in general

Answer 28

All show vascular injury with mural necrosis and haemorrhage Localised, organ restricted or systemic Some are granulomatous Secondary ischaemia of down-stream tissue May cause infarction

Question 29

Polyarteritis nodosa

Answer 29

Systemic vasculitis Small/medium sized arteries (not veins or smaller vessels) Often spares lungs Segmental necrotising inflammation of arteries especially renal, cardiac and GI tract Branching sites particularly

Question 30

Polyarteritis nodose diagnosing

Answer 30

Angiogram | See hallmark changes- usually aneurysms

Question 31

Polyarteritis nodose can lead to

Answer 31

Aneurysms, infarcts, haemorrhage Microscopically transmural inflammation and fibrinoid necrosis Possible thrombosis of vessel After healing, there is fibrosis which later becomes nodular Lesions of different ages

Question 32

Polyarteritis nodosa features

Answer 32

``` Young adults More men than women Acute, subacute or chronic Fever, malaise, weight loss, HT abdo pain + melaena, muscular pains, renal involvement (not gloms), peripheral neuritis 30% have Hep B antigen + Therapy- steroids + cyclophosphamide ```

Question 33

ANCA related vasculitis

Answer 33

Systemic/renal limited/other 85% ANCA positive Flu-like illness Fever, arthralgia, myalgia, purpura, peripheral neuropathy, GI involvement May be provoked by drugs (propylthiouracil, penicillamine, hydralazine)

Question 34

ANCA related vasculitis staining patterns

Answer 34

cytoplasmic and perinuclear (c and p) by IMF c-ANCA- targeting proteinase r (PR3) p-ANCA- targeting myeloperoxidase (MPO)

Question 35

ANCAs in patients without vasculitis

Answer 35

Non-MPO p-ANCA in UC, PSC, autoimmune hep, RA/Felty's

Question 36

Wegener's granulomatosis (aka granulomatosis with polyangitis)

Answer 36

``` Upper/lower RT Eyes/ears Necrotising granulomas Vasculitis c-ANCA usually Proteinase 3 (PR3) Often have kidney involvement ```

Question 37

ANCA treatment

Answer 37

NEED TO BE TREATED- Untreated 80% 1 year mortality Treated 75% 5 year survival Aggressive immunosuppression with cyclophosphamide + steroids

Question 38

Cardiomyopathy

Answer 38

Cardiac disease resulting from primary intrinsic myocardial abnormality Exclude other causes e.g. ischaemia Causes are idiopathic or secondary to known cause 3 types (mainly involving left ventricle)- dilated, hypertrophic + restrictive 4th type- arrhythmogenic right ventricular CM

Question 39

Dilated cardiomyopathy

Answer 39

Progressive cardiac dilatation + contractile dysfunction Big heart 4 chamber dilatation Valves/arteries not significantly abnormal May be thrombi Microscope -myocardial hypertrophy/fibrosis

Question 40

Dilated cardiomyopathy features

Answer 40

``` Idiopathic Genetic 930%) Post myocarditis Alcohol or other toxicity e.g. doxorubicin Pregnancy associated Haemochromatosis Sarcoidosis ```

Question 41

Dilated cardiomyopathy patients

Answer 41

``` any age but usually 20-50 Progressive CCF Signs of LVF + RVF Death from failure or sudden death (arrhythmia) Need transplant ```

Question 42

Hypertrophic cardiomyopathy

Answer 42

Hypertrophic heart Poor diastolic filling Often outflow obstruction Little or no dilatation Classically disproportionate thickening of septal myocardium esp. subaortic Microscope- hypertrophy, disarray + fibrosis

Question 43

Hypertrophic cardiomyopathy features

Answer 43

Mutation of muscle protein esp. beta-myosin heavy chain Most familial Many diff. mutations Leads to poor compliance + reduced LV chamber size +/- outflow obstruction Clinically heterogenous, may need surgery to remove muscle in LV and let blood flow through

Question 44

Restricted cardiomyopathy

Answer 44

Primary decrease in ventricular compliance Idiopathic Secondary to irradiation fibrosis, amyloid, sarcoid, tumour metastases, other Firm ventricles (otherwise normal) with dilated atria Microscopy may reveal cause

Question 45

Myocarditis

Answer 45

Inflammation causing myocardial injury and not a response to it Infections- viruses (most common) e.g. Coxacjieviruses, enteroviruses, HIV; chlamydia, rickettsiae, bacteria, fungi, protozoa, Helminths Immunological- post-viral, SLE, drug reactions, transplant rej Others- sarcoidosis, giant cell mypcarditis

Question 46

Myocarditis injury

Answer 46

May be direct damage or T cell mediated injury to antigens on myocyte surface Inflammation and myocyte necrosis Clinically fatigue, fever, chest discomfort, HF, arrhythmias, sudden death May mimic acute infarct May lead to dilated cardiomyopathy