Pulmonary HTN Drugs

Question 1

When do you use a CCB to treat PH?

Answer 1

if positive vasopressor test

Question 2

What is the definition of PAH?

Answer 2

sustained elevation of mPAP to 25 mmHg or greater

Question 3

Who is the typical pt w/ PAH?

Answer 3

young mother

more common in females, can occur at any age

Question 4

What is the significance of BMPR2?

Answer 4

first gene linked to PAH, but seen in <25% of idiopathic PAH

gene product is supposed to be a brake to vasoconstriction system

Question 5

Generally, what is the cellular theory of development of PAH?

Answer 5

messed up K+ channels –> more calcium in cell –> contraction, proliferation, and apoptosis

serotonin and endothelin I further increase calcium to make this worse

Question 6

What can you see on CXR in PAH?

Answer 6

peripheral hypovascularity in lungs

prominent central pulmonary A

RV enlargement

Question 7

What do you see on ECG in PAH?

Answer 7

right axis deviation of QRS complex

Question 8

What is the vasopressor test for PAH?

Answer 8

short-acting vasodilator given –> positive if PaP falls 10 or more, mean pulm A pressure drops 40 or more, CO is unchanged or increased

+ test can signal you to prescribe non-dyh CCBs

If negative –> don’t use CCBs

Question 9

What are the extra txs besides specific PAH meds?

Answer 9

anticoagulants

diuretics

Oxygen therapy

Question 10

How are prostacyclin analogs given?

Answer 10

continuous IV, subcutaneous infusion or intermittent nebulizer

Question 11

What are the 4 basic groups of PAH meds?

Answer 11

prostacyclin analogs

endothelin receptor antagonists

PDE-5 inhibitors

soluble guanylyl cyclase stimulants

Question 12

How is epoprostenol given?

Adverse effects?

Answer 12

continuous IV that has to be kept cold (short half-life)

sepsis due to chronic catheter

nausea and vomiting, HA, flushing, jaw pain

Question 13

what are the 3 benefits prostacyclin analogs have?

Answer 13

promotes vascular relaxation

suppresses growth of vasc smooth muscle cells

inhibits platelet aggregation

Question 14

How is treporstinil given?

Side effects?

Answer 14

subcut infusion, but painful –> now often diluted and admin w/ pump IV (like epoprostenol)

longer half life and no refrigeration

S/e similar to that of epoprostenol (sepsis, general systemic sx)

Question 15

How is iloprost given?

Side effects?

Answer 15

inhalation 6-9 times/day

severe: fainting due to hypotension, cough, HA, flushing, jaw pain

Question 16

How is selexipag given?

Answer 16

admin orally BID = easier than other prostanoids, but super expensive!

Question 17

what is the suffix for endothelin antagonists?

Answer 17

-entan

(bosentan, ambrisentan, and macitentan)

Question 18

What is the word root for prostanoids and what are those drugs?

Answer 18

-prost

epoprostenol, treprostinil, iliprost, selexipag (exception to word rule)

Question 19

What is the half life of sildenafil?

Answer 19

~4 hrs

tadalafil has longer

Question 20

What is riociguat’s MOA?

Answer 20

soluble guanylate cyclase sensitizer –> directly stimulates independent of NO –> vasodilation

increases generation of cGMP –> incr vasodilation

Question 21

What is the effect of all PAH drugs except prostanoids?

Answer 21

improves exercise tolerance and slows sx progression

Question 22

What is the MOA of bosentan?

adverse effects?

Answer 22

non-spec block of ETa and ETb endothelin receptors

hepatotoxicity, teratogenesis

accelerates metabolism of warfarin and oral contraceptives (must use 2 forms of birth control)

Question 23

How are endothelin antagonists given?

Answer 23

orally

Question 24

What is the MOA of ambrisentan?

Adverse effects?

Answer 24

selectively blocks Eta receptors that cause vasoconstriction and promote cell proliferation

teratogensis, *but doesn’t damage liver (vs bosentan)

*doesn’t accelerate metabolism of warfaran and Oral contraceptives vs bosentan

but super expensive!

Question 25

What distinguishes macitentan from other endothelin antagonists?

Answer 25

18 hr half life that permits pid dosing

Question 26

How is riociguat given?

What should it not be given with?

Answer 26

oral - half life ~ 12 hrs

don’t give w/ NO donors or PDE-5 inh

teratogen potential