Pulmonary hypertension

Question 1

Definition of pulmonary hypertension?

Answer 1

Increase in mean PAP greater than 25mmHg, as assessed by right heart catheterization

Question 2

How does pulmonary hypertension present? (3)

Answer 2

Progressive shortness of breath
Tiredness
Dizziness/syncope

Question 3

Clinical signs of PAH?

Answer 3

Elevated JVP
Peripheral oedema
Right parasternal heave
Loud P2

Question 4

WHO classification of PAH?

Answer 4

Group 1- PAH (idiopathic, familial, associated with other diseases)
Group 2- associated with left heart disease
Group 3- associated with lung disease/hypoxaemia
Group 4- due to chronic thromboembolic disease
Group 5- miscellaneous

Question 5

Examples of lung conditions which can lead to pulmonary hypertension?

Answer 5

COPD
Pulmonary fibrosis
Living at altitude
Obstructive sleep apneoa

Question 6

Pulmonary hypertension can occur in what group of diseases?

Answer 6

Connective tissue diseases e.g. scleroderma, SLE, RA

Question 7

Drugs used to treat pulmonary hypertension?

Answer 7

Sildenfail (PDE-5 inhibitor)

Bosentan (Endothelin receptor antagonist)