Pulmonary HTN Drugs Flashcards
When do you use a CCB to treat PH?
if positive vasopressor test
What is the definition of PAH?
sustained elevation of mPAP to 25 mmHg or greater
Who is the typical pt w/ PAH?
young mother
more common in females, can occur at any age
What is the significance of BMPR2?
first gene linked to PAH, but seen in <25% of idiopathic PAH
gene product is supposed to be a brake to vasoconstriction system
Generally, what is the cellular theory of development of PAH?
messed up K+ channels –> more calcium in cell –> contraction, proliferation, and apoptosis
serotonin and endothelin I further increase calcium to make this worse
What can you see on CXR in PAH?
peripheral hypovascularity in lungs
prominent central pulmonary A
RV enlargement
What do you see on ECG in PAH?
right axis deviation of QRS complex
What is the vasopressor test for PAH?
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
What are the extra txs besides specific PAH meds?
anticoagulants
diuretics
Oxygen therapy
How are prostacyclin analogs given?
continuous IV, subcutaneous infusion or intermittent nebulizer
What are the 4 basic groups of PAH meds?
prostacyclin analogs
endothelin receptor antagonists
PDE-5 inhibitors
soluble guanylyl cyclase stimulants
How is epoprostenol given?
Adverse effects?
continuous IV that has to be kept cold (short half-life)
sepsis due to chronic catheter
nausea and vomiting, HA, flushing, jaw pain
what are the 3 benefits prostacyclin analogs have?
promotes vascular relaxation
suppresses growth of vasc smooth muscle cells
inhibits platelet aggregation
How is treporstinil given?
Side effects?
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)
How is iloprost given?
Side effects?
inhalation 6-9 times/day
severe: fainting due to hypotension, cough, HA, flushing, jaw pain
How is selexipag given?
admin orally BID = easier than other prostanoids, but super expensive!
what is the suffix for endothelin antagonists?
-entan
(bosentan, ambrisentan, and macitentan)
What is the word root for prostanoids and what are those drugs?
-prost
epoprostenol, treprostinil, iliprost, selexipag (exception to word rule)
What is the half life of sildenafil?
~4 hrs
tadalafil has longer
What is riociguat’s MOA?
soluble guanylate cyclase sensitizer –> directly stimulates independent of NO –> vasodilation
increases generation of cGMP –> incr vasodilation
What is the effect of all PAH drugs except prostanoids?
improves exercise tolerance and slows sx progression
What is the MOA of bosentan?
adverse effects?
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)
How are endothelin antagonists given?
orally
What is the MOA of ambrisentan?
Adverse effects?
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!
What distinguishes macitentan from other endothelin antagonists?
18 hr half life that permits pid dosing
How is riociguat given?
What should it not be given with?
oral - half life ~ 12 hrs
don’t give w/ NO donors or PDE-5 inh
teratogen potential
