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!
