Pulmonary Vascular Diseases

Question 1

What are the types of pulmonary vascular diseases?

Answer 1

Embolism

Hypertension

Question 2

Pulmonary embolism risk factors?

Answer 2

Recent major trauma
Recent surgery
Cancer
Significant cardiopulmonary disease e.g. MI
Pregnancy
Inherited thrombophilia

Question 3

Symptoms of PE

Answer 3

Pleuritic chest pain
Cough
Haemoptysis
Isolated acute SOB
Syncope or cardiac arrest

Question 4

Signs of PE

Answer 4

Pyrexia
Pleural rib
Stony dullness at base (pleural effusion)
Tachycardia
Tachypnoea
Hypoxia
Hypotension

Question 5

Possible investigations in suspected PE?

Answer 5

FBC and biochem
Blood gases
CXR
ECG

D-dimer test
CTPA
V/Q Scan

EchoCG
Consider CT abdomen and mammography
Consider thrombophilia testing

Question 6

How can PE be scored?

Answer 6

Wells score - include symptoms and signs of VTE, previous VTE and risk factors

Revised Geneva Score
- based on risk factors, symptoms and signs

Question 7

How would you treat a PE?

Answer 7

O2
LMWH
Warfarin
Direct oral anticoagulants
- rivaroxaban, apixaban
Thrombolysis
- alteplase (rt-PA)
Pulmonary embolectomy

Question 8

What is the prognosis in a PE?

Answer 8

Mortality after 30 days varies from 0-25%
PESI score
- based on age, sex, comorbidities, and physiological parameters

Question 9

What is the definition of pulmonary hypertension?

Answer 9

Elevated BP in the pulmonary arteries or tree
Defined as MAP (pulmonary) of >25mmHg
Either primary or secondary

Question 10

Risk factors for pulmonary hypertension?

Answer 10

Idiopathic
Secondary to chronic respiratory disease
Secondary to left heart disease
Chronic thromboembolic PH
Miscellaneous
- collagen vascular disease
- portal hypertension
- congenital heart disease (L to R shunt)
- HIV

Untreated, rapidly progressive and leads to death
Primary PAH is rare (15-50/million population)

Question 11

Symptoms of pulmonary hypertension?

Answer 11

Exertional SOB
Chest tightness
Exertional presyncope or syncope

Question 12

Signs of pulmonary hypertension?

Answer 12

Elevated JVP
RV heave
Loud pulmonary second heart sound
Hepatomegaly
Ankle oedema

Question 13

What investigations might you do in pulmonary hypertension?

Answer 13

ECG
Lung function tests
CXR

EchoCG
V/Q scan
CTPA
Right heart catheterisation
- allows direct measure of pulmonary arterial pressure
- measurement of wedge pressure
- measurement of cardiac output

Question 14

What are the treatments for pulmonary hypertension?

Answer 14

General treatments:

• treat underlying conditions
• O2
• anticoagulants
• diuretics

Specific:
- CCB (amlodipine)
- prostacyclin
- endothelin receptor antagonists (bosentan, ambrisentan)
PDE inhibitors (sildenafil, tadalafil)

Other treatments:

• thromboendarterectomy (CTEPH)
• lung/heart transplant