Pulmonary Hypertension

Question 1

What is it?

Answer 1

Mean pulmonary artery pressure of 25mmHg or more at rest and 30mmHg at exercise

Increased vascular resistance results in right ventricular failure

Question 2

What are the classifications?

Answer 2

1) Idiopathic- familial, HIV,
2) Present with left sided heart disease
3) Resp disease e.g. COPD, ILD, OSA
4) Thromboembolic disease e.g. PE
5) Miscellaneous- sarcoidosis

Question 3

What are clinical features?

Answer 3

SoB- progressive exertional dyspnoea
Features of RHF

O/E
- left parasternal heave
- Loud P2
- Raised JVP
- Tricuspid regurg- pansystolic murmur

Question 4

What investigations are required?

Answer 4

Echo
Right heart catheterisation to confirm

Question 5

What is the management?

Answer 5

Treat underlying cause

Perform acute vasodilator testing
- assess who may respond to CCBs
- monitor pulmonary artery pressures following vasodilator administration
- +ve response= drop in 10mmHg

Medical
- CCB e.g. nifedipine if +ve response
- Negative response e.g. iloprost, bosentan, slidenafil