Pulmonary Arterial Hypertension

Question 1

What are the 3 different classifications of PAH?

Answer 1

Idiopathic
Familial
Secondary

Question 2

What causes familial PAH?

Answer 2

Mutation in BMPII receptors

Question 3

What causes secondary PAH?

Answer 3

Pulmonary fibrosis
COPD
Altitude
Any other condition leading to chronic hypoxia

Question 4

How is PAH treated?

Answer 4

Endothelin receptor antagonists (ERA)
Prostanoids (prostacyclin analogues)
PDEI

Question 5

Bosentan

Answer 5

Non-selective ERA

Question 6

Sildenafil

Answer 6

PDE V inhibitor

Used in PAH and erectile dysfunction

Question 7

What is the effect of PAH on RA and systemic venous pressures?

Answer 7

They are increased

Question 8

What is the effect of increased systemic venous pressure on the systemic capillary bed?

Answer 8

Peripheral oedema
Ascites
Pleural effusions

Question 9

What are the 3 main causes of secondary pulmonary hypertension? Which is most common?

Answer 9

Increased LAP
Increased pulmonary blood flow
Increased pulmonary vascular resistance (most common)

Question 10

What are 3 causes of increased pulmonary vascular resistance?

Answer 10

Vasoconstriction (e.g. low PAO2)
Obstruction (e.g. PE)
Obliteration (e.g. emphysema)