Thoracic Aortic Disease & Vasculitides

Question 1

What are the different locations or classes of thoracic aneurysm? [3]

Answer 1

Ascending Aorta

Aortic Arch

Descending Aorta (After that your into Abdominal Aorta)

Question 2

What are the types of aneurysm? [2]

Answer 2

True & False True aneurysms are split into saccular & fusiform

Question 3

What is a true aneurysm? [1]

Answer 3

Where all 3 layers of aortic wall are dilated.

One side = Saccular

All around = Fusiform

Question 4

What is a false aneurysm? [2]

Answer 4

Some or all of the wall layers rupture. [1]

The haematoma is contained by the intact adventitia or surrounding soft tissue [1]

Question 5

What causes an aneurysm? Differentiate between true [5] and false [3]

Answer 5

True:

• • Atherosclerosis
• • Connective Tissue Diseases (e.g. Marfan’s)
• • Trauma
• • Congenital Bicuspid Aortic Valve
• • Infections e.g. Syphilis

False:

• • Inflammatory Conditions (E.g. Takayasu’s Arteritis)
• • Trauma
• • Iatrogenic

Question 6

Risk factors for an aneurysm [5]

Answer 6

Age

Smoking

Marfan’s etc.

Family History

Hypercholesterolaemia

Hypertension

Question 7

What are the symptoms of a thoracic aortic aneurysm? [6]

Answer 7

They vary with location and are often asymptomatic: [1]

• • SOB
• • HF (if causing AR)
• • Dysphagia/Hoarseness (If chronic asc. aortic aneurysm)
• • Sharp Chest Pain radiating to Central upper back (spreads if dissection)
• • Regional Hypotension

Question 8

What are the clinical signs of a FALSE aneurysm? [3]

Answer 8

Thrill - Bruit - Pulsatile mass

Question 9

What are the possible outcomes/complications of an aneurysm? [3]

Answer 9

1. Rupture
2. Ischaemia/Infarction
3. Distal Emboli causing their own infarctions

Question 10

What is Takayasu’s Arteritis? [3]

Answer 10

Takayasu’s arteritis is a large vessel granulomatous vasculitis.[1] It typically causes occlusion of the aorta and questions commonly refer to an absent limb pulse. [1] It is more common in females and Asian people [1]

Question 11

Presentation of takayasu’s arteritis [5]

Answer 11

1. systemic features of a vasculitis e.g. malaise, headache
2. unequal blood pressure in the upper limbs
3. carotid bruit
4. intermittent claudication
5. aortic regurgitation (around 20%)

Question 12

How do we treat Takayasu’s Arteritis? [2]

Answer 12

Steroids & Surgery

Question 13

Investigation of TAAs [5]

Answer 13

Bloods:

• FBC, clotting, U&E and creatinine, CRP, LFTs, amylase, syphilis and HIV serology, connective tissue disease genetic testing

ECG

Echo:

• TTE to view aortic valve and root, TOE to view from valve to proximal descending aorta

USS abdo:to look for associated abdo aneurysms

CT with contrast

Question 14

Management of TAAs [5]

Answer 14

Surgery :thoracotomy with Dacron graft +/- aortic root or valve replacement

Medical:

• 6 monthly CT or MRI of aneurysm
• BP control with beta blockers (reduce shear stress across aortic wall)
• smoking cessation
• mx of underlying cause, mx of other cardiac RFs

Question 15

What are indications for surgical intervention of TAA [2]

Answer 15

symptomatic or asymptomatic and >5.5cm (ascending aorta) or >6cm (descending aorta)

Question 16

In TAA ruptures, what can happen and how will this affect clinical presentation [6]

Answer 16

Erosion into nearby structures:

• Haematemesis (aorto-oesophageal fistula)
• Haemoptysis (aorto-bronchial fistula)
• Haemothorax
• Cardiac tamponade (ruptures into pericardium)
• Acute L>R shunt (aorto-pulmonary artery fistula)

Question 17

Management in TAA rupture [4]

Answer 17

IV access and bloods:FBC, clotting, U&E and creatinine, LFT, Cross Match

Immediate surgical repair:

• thoracotomy with Dacron graft +/- aortic root
• or valve replacement
• aim is the secure proximal aortic control without disturbing any tamponade effect provided by extra-aortic structures or haematoma

Question 18

Vasculitides are categorised into large vessel [2], medium vessel [2] and small vessel [3]

Answer 18

Large vessel

• temporal arteritis
• Takayasu’s arteritis

Medium vessel

• polyarteritis nodosa
• Kawasaki disease

Small vessel

• ANCA-associated vasculitides (Wegener’s*, Churg-Strauss*, microscopic polyangiitis)
• Henoch-Schonlein purpura
• cryoglobulinaemic vasculitis

Question 19

What are the vasculitides?

Answer 19

The vasculitides are a group of conditions characterised by inflammation of the blood vessel walls. This may, in turn, compromise vessel integrity. Constitutional symptoms may be present.

Question 20

Buergers disease definition [2]

Epidemiology

Presentation [2]

Investigation

Answer 20

Affects large and medium sized arteries. Segmental thrombotic occlusions of lower limb vessels

Ep: Commonest in young male smokers

Presentation

• Proximal pulses usually present, but pedal pulses are lost
• An acuter hypercellular occlusive thrombus is often present

Ix: Tortuous corkscrew shaped collateral vessels may be seen on angiography

Question 21

Giant cell arteritis definiton [1]

Epidemiology

Which is the most commonest type?

Investigation

Answer 21

Systemic granulomatous arteritis that usually affects large and medium sized vessels

Ep: F>M

Temporal arteritis is commonest type

Granulomatous lesions may be seen on biopsy (although up to 50% are normal)

Question 22

Polyarteritis nodosa definition [1]

Epidemiology/risk factor

Associated with…

Ix

Answer 22

Systemic necrotising vasculitis affecting small and medium sized muscular arteries

Most common in populations with high prevalence of hepatitis B

Renal disease is seen in 70% cases

Angiography may show saccular or fusiform aneurysms and arterial stenoses

Question 23

Wegeners granulomatosis definition

Which classification of vasculitides is this in?

Presentation

Investigation

Answer 23

Systemic necrotising granulomatous vasculitis

Predominantly affects small and medium sized arteries

Presentation: Cutaneous vascular lesions may be seen (ulceration, nodules and purpura)

Ix: Sinus imaging may show mucosal thickening and air fluid levels