Pulmonary Arterial Hypertension

Question 1

How many groups of pulmonary HTN are there?

Answer 1

5 groups

Question 2

Group 1 pulmonary HTN features

Answer 2

Group 1
Pre-capillary pulm HTN
Includes:
- Idiopathic/heritable (primary)
- Associated with other diseases e.g. CTD (secondary)
Rare
Usually treated with medical therapy (PAH drugs, CCB in responders), lung transplant

Question 3

Group 2 pulmonary HTN features

Answer 3

Group 2
Pulm HTN relating to L heart disease
Includes:
- IpcPH
- CpCPH
The most common cause of pulm HTN
Mainstay of treatment is treatment of the underlying L heart disease - usually NOT treated with pulmonary vasodilators unless have disproportionally high pulmonary vascular resistance

Question 4

Group 3 pulmonary HTN features

Answer 4

Group 3
Pulm HTN relating to underlying lung disease and hypoxia
i.e. Cor pulmonale , RH failure relating to lung disease
Includes
- Non-severe PH
- Severe PH
Common
Mainstay of treatment is treatment of underlying lung disease (also some emerging trial evidence for inhaled treprostinil)

Question 5

Group 4 pulmonary HTN features

Answer 5

Group 4
Pulm HTN due pulmonary artery obstruction ( most commonly chronic thromboembolic disease)
i.e. sequelae of acute PE where blood clot does not resolve and causes vascular occlusion resulting in RH failure
Rare
CANNOT afford to miss this group as there are surgical interventions which can be offered e.g. PEA, interventional radiology interventions

Question 6

Group 5 pulmonary HTN features

Answer 6

Group 5
Pulm HTN with unclear and/or multifactorial mechanism
E.g. haematologic disorders, systemic disorders e.g. sarcoidosis
Rare