mPAP =
> = 25 mmHg
medications that cause PAH
cocaine
fenfluramine
methamphetamine/ amphetamine
SSRI use during pregnancy increase risk of newborn
weight loss drugs
pathophys
increase vasoconstrictor (endothelin-1, TXA2)
decrease of vasodilator (prostacyclins)
diagnosis
right heart cauterization
short acting vasodilators given for vasoreactivity testing
what is pulmonary fibrosis
scarred and damage lung tissue + SOB, cough
med induced pulmonary fibrosis
amiodarone/ dronedarone
bleomycin
busulfan
carmustine/ lomustine
medications approved for PF treatment
pirfenidone (erbriet)
nintedanig (ofev)
non pharm
NA+ restrictions <2.4 g qd
avoid nsaids
influenza + pneumonia vaccine
avoid high altitudes
oxygen supplementation
if responder tx
PO CCB
If non responder or failed ccb tx
prostacyclin analog & receptor agonist
ERAs
PDE5i
sGC stimulators
supportive tx
loop
digoxin
Anticoagulant, if warfarin (int 1.5-2.5)
epoprostenol notes
vasodilation reactions
rebound HTN if interruption
infusion pain
must protect from light
reconstitution require ice pack for stability
endothelin receptor antagonist
teronogenic
hepatotoxicity
REMS program
PDE5 i
avoid use with nitrates
hearing loss
vision loss
hypotension
priapism
headache
sGC (riociguat)
teratogenic
REMS program
hypotension
HA
CI with nitrates and PDE5i
