Pulmonary vascular disease: pulmonary hypertension

Question 1

What is it?

Answer 1

Increased pressure in the pulmonary artery due to thickening of capillaries and arterioles.
Defined as being a pressure greater than 25mmhg

Question 2

Causes

Answer 2

1- Idiopathic-very rare-1 or 2 per million- very aggressive
2- Secondary to chronic heart disease
3-secondary to left heart disease
4-Chronic thromboembolic pulmonary hypertension 
5-Miscellaneous:
-HIV
-portal hypertension
-heart shunt
-collagen vascular heart disease

Question 3

Symptoms

Answer 3

• Shortness of breath- dyspnoea
• presyncope (lightheadness) or syncope (loss of consciousness)
• chest pain

Question 4

signs

Answer 4

Early:
-elevated jugular venous pressure
-right ventricular heave-obstruction
Later:
ankle oedema 
Liver enlargement=hepatomegaly

Question 5

Investigations

Answer 5

• ECG
• Echocardiogram-definite test as allows measurement of pulmonary artery pressure when leak in tricuspid valve
• V/Q scan
• Chest X-ray
• Lung function test
• CT pulmonary angiogram
• right heart catheritisation: pulmonary artery pressure/ wedge pressure/cardiac output

Question 6

Treatment

Answer 6

• o2 if related to hypoxia
• diuretics to relieve swelling
• anticoagulants if related to pulmonary embolism

Question 7

specific treatments

Answer 7

• prostacyclin
• Calcium channel antagonist
• Phosphodiesterase inhibitors
• endothelin receptor antagonist