Pulmonary arterial hypertension Flashcards
PH is defined as
> = 25mmHg
Key drugs that can cause PAH
- cocaine
- SSRI use during prego (increased risk of persistent pulmonary hypertension of a newborn)
- Weight-loss drugs (diethylpropion, phendimetrazine, phentermine)
- methamphetamine/amphetamines
responder
CCB tx 10%
verapamil is not recommended due to it’s more pronounced inotropic effects compared to diltiazem
generally long-acting CCB so long-acting nifedipine, diltiazem, and amlodipine
non-responder to vasoreactivity test
- PDE-5 inhibitors
- endothelin receptor antagonists
- soluble guanylate cyclase (sGC) stimulator
- prostacyclin analogue
support PAH therapies
similair to systolic HF
- loop diuretics
- digoxin
- if pro-thrombotic stat3e, warfarin INR 1.5-2.5 and if it is chronic thromboembolic pulmonary hypertension INRO 2-3
prostacyclin analogues and receptor agonists
- potent vasodilators and inhibitors of platelet aggregation
- Epoprostenol and treprostinil can be admin by continuous IV infusion at home using ambulatory infusion pump
- treprostinil- increased by cyp2C8 inhibitors (gemfibrozil) and decrease w/ inducers (rifampin)
- flolan, veletri, remodulin are very potent vasodilators, avoid interruption and sudden, large dose reduction
epoprostenol
Flolan, veletri ng/kg/min
continuous IV infusion
protect from light before constitution and during infusion
reconstitution solutions of Flolan requires use of ice packs for stability
short t1/2- 6 mins
contra: HF w/ decreased left ventricular EF
- prostacyclin analogs
Treprostinil
remodulin ng/kg/min
tyvaso: inhalation
orenitram: oral ER tab
contra: oral tx contra in severe hepatic impairment child-pugh class C
ghost tablet- oral
t1/2- 4 hrs
- prostacyclin analogs
illoprost
ventavis inhalation
- prostacyclin analogs
selexipag
uptravi
- prostacyclin receptor agonist
prostacyclin analogues and receptor agonists
safety/SE/monitoring
- vasodilation reaction (hypotension, flushing, heachache, dizziness)
- rebound PH
SE: hypotension, flushing, jaw pain
endothelin receptors antagonist
- block endothelial receptors
- ambrisentan 5mg when given with cyclosporines
- levels of bosentan can increase w/ cypinhibitors
- bosentan can decrease effectiveness of hormonal contraceptive
bosentan
tracleer
- teratogenic (neg prego test before and every month) REMS
- hepatotoxic! liver failure
- decrease in Hgb/Hct
- contra: prego, cyclosporine or glyburide
- hypersensitivity reaction (DRESS, etc.)
SE: headache, upper respiratory infection
>3 older
- ERA antagonist
ambrisentan
letairis
- REMS teratogenic prego, nego prego test..
- contra: prego, idiopathic pulmonary fibrosis
- hepatotoxic, Hgb/Hct
SE: headache, upper respiratory infection
- ERA antagonist
macitentan
osempit
- REMS teratogenic prego, nego prego test..
- contra: prego
- hepatotoxic, Hgb/Hct
SE: headache, upper respiratory infection
- ERA antagonist
