Pulmonary Arterial Hypertension Flashcards
(30 cards)
PH
continuous high BP in pulmonary arteries
meds that cause PH
codeine
SSRI during pregnancy inc risk of persistent PH of newborn (PPHN)
weight-loss: diethylpropion, phendimetrazine, phentermine)
methamphetamines/amphetamines
others: dasatinib, diazoxide, fenfluramine
pathophysiology of PH
imbalance of vasoconstrictor and vasodilator
vasoconstrictor: endothelin-1 and thromboxane A2 (TXA2)
vasodilator: prostacyclines
HF is most common cause of death in PAH
lung/heart-lung transplant may be option for younger patients
symptoms of PAH
fatigue, dyspnea
non-drug treatment of PAH
sodium restrict to <2.4 g/day
avoid NSAIDs and other Na/H2O-retention meds
routine flu and pneumococcal shots
drug treatment of PAH
confirm diagnosis with right heart catheterization (vasoreactivity tested)
if responder (mPAP drops >=10 and to <40) - give CCB (long-acting nifedipine, diltiazem, and amlodipine); do not use verapamil bc negative inotrope
if non-responder: vasodilators (prostacyclin analogs and receptor agonists, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, soluble guanylate cyclase stimulator; IV epoprostenol dec mortality and first line
supportive therapies: loops for volume overload; digoxin for CO/control HR; drugs for pro-thrombotic state - warfarin with INR goal 1.5-2.5
prostacyclin analogues (prostanoids) and receptor agonists
epoprostenol, treprostinil, remodulin, iloprost, selexipag
potent vasodilators and inhibitors of platelet aggregation
continuous IV at home
epoprostenol dosing
continuous IV
2 ng/kg/min
treprostinil dosing
Remodulin: continuous SC or IV infusion ng/kg/min
prostacyclin analogues warnings
vasodilation (hypotension, flushing)
rebound PH
chronic iv infusions: infections
prostacyclin analogues side effects
hypotension
flushing
jaw pain
infusion-site pain
prostacyclin anagloues notes
parenteral agents: potent vasodilators; avoid interruptions and sudden, large dose reductions
epoprostenol: must protect from light during infusion
Flolan: reconstituted solution require ice packs
endothelin receptor antagonists
bosentan, ambrisentan, macitentan
endothelin receptor antagonists BBW
teratogenic: must have negative pregnancy test before starting and monthly during
Bosentan: hepatotoxicity
REMS program only
endothelin receptor antagonists CI
pregnancy
endothelin receptor antagonists warnings
hepatotoxicity
dec Hbg/Hct
fluid retention
endothelin receptor antagonists side effects
headache
endothelin receptor antagonist DI
Bosentan: dec effectiveness of hormonal contraceptive
PDE-5 inhibitors
inc cGMP = pulmonary vasculature relaxation and vasodilation
sildenafil, tadalafil
PDE-5 inhibitors CI
nitrates
PDE-5 inhibitors warnings
hearing loss
vision loss (NAION)
hypotension
priapism
PDE-t inhibitors side effects
headache
PDE-5 inhibitor DI
do not use with nitreates = low BP; absolute CI
caution with alpha-blocker (or other antihypertensives) = hypotension
soluble guanylate cyclase stimulator
guanylate cyclase is receptor for endogenous NO
Riociguat sensitizes sGC and directly stimulates = inc cGMP = relaxation of pulmonary artery smooth muscle cells
