Pulmonary hypertension

Question 1

What are the groups of pulmonary hypertension?

Answer 1

Group 1 - pulmonary arterial hypertension
Group 2 - left heart disease
Group 3 - lung disease
Group 4 - CTEPH
Group 5 - miscellaneous

Question 2

A mean pulmonary artery pressure ≥(a) is considered diagnostic for pulmonary hypertension. The other pressure measurement taken during RHC is the (b), which is an estimate for (c) pressure. These two measurements along with cardiac output are used to calculate (d), which is represented in (e) units.

Answer 2

a) 20-25
b) Pulmonary capillary wedge pressure
c) Left atrial pressure
d) Pulmonary vascular resistance
e) Woods

Question 3

What mPAP, mPCWP, and PVR on RHC would be suggestive of isolated pre-capillary pulmonary hypertension?

Answer 3

mPAP > 20-25
mPCWP < 15 (>15 suggests LHD as cause for pulmonary HTN)
PVR > 3

Question 4

What measurement on TTE is used to estimate pulmonary arterial systolic pressure?

Answer 4

Tricuspid regurgitant jet velocity

Question 5

What are the four classes of drugs used to treat pulmonary arterial hypertension?

Answer 5

Calcium channel blockers in positive responsders to vasoreactivity testing

Endothelin receptor antagonists (ERAs)

PDE5 inhibitors - sildenafil, riociguat

Prostanoids (synthetic prostacyclin) - iloprost, epoprostenol

Question 6

A positive response to a vasoreactivity test on right heart catheterisation involves a reduction in (a) by ≥_(b)_mmHg.

Answer 6

a) mPAP

b) 10

Question 7

Name five causes of pulmonary arterial hypertension.

Answer 7

• Idiopathic
• Familial (most common mutation BMPR2)
• Connective tissue disorders e.g. scleroderma, SLE
• Congenital heart disease
• Portal hypertension
• Drugs (e.g. amphetamines)
• HIV