Pulmonary HTN and cor pulmonale

Question 1

How do you manage pulmonary HTN first?

Answer 1

REFER to pulmonologist (1st) and cardiologist (2nd)

Question 2

vasodilators for treating Group 1 pulm HTN that is unable to be treated with CCBs or NO

Answer 2

• Nitric oxide pathway w/ phosphodiesterase inhibitors (sildenafil, tadalafil)
• soluble guanylate cyclase stimulators (riociguat)
• Endothelin pathway: endothelin receptor antagonists (bosentan, ambrisentan, macitentan)
• Prostacyclin pathway: IV epoprostenol, treprostinil, inhaled iloprost, selexipag

Question 3

Tx: group 1 (responsive) pulm HTN

Answer 3

CCBs

Question 4

tx: group 2 and 3 pulm HTN

Answer 4

Treat underlying conditions
NO PAH-specific therapies

Question 5

tx: group 4 pulm HTN

Answer 5

Treat w/ lifelong anticoagulation
Pulmonary endarterectomy (PEA) w/ surgically accessible CTEPH when possible
OR balloon pulmonary artery angioplasty in ineligible
Avoid CCBs

Question 6

tx: group 5 pulm HTN

Answer 6

Treat underlying disorder

Question 7

How do you treat cor pulmonale?

Answer 7

Treat COPD - oxygen therapy

Vasodilators – may not help w/ COPD-related, + inhalations of NO to O2 therapy to decrease PAP

Exclude other causes, try clinical trials

Diuretics (furosemide)