Pulmonary Arterial Hypertension

Question 1

PH

Answer 1

continuous high BP in pulmonary arteries

Question 2

meds that cause PH

Answer 2

codeine
SSRI during pregnancy inc risk of persistent PH of newborn (PPHN)
weight-loss: diethylpropion, phendimetrazine, phentermine)
methamphetamines/amphetamines
others: dasatinib, diazoxide, fenfluramine

Question 3

pathophysiology of PH

Answer 3

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

Question 4

symptoms of PAH

Answer 4

fatigue, dyspnea

Question 5

non-drug treatment of PAH

Answer 5

sodium restrict to <2.4 g/day
avoid NSAIDs and other Na/H2O-retention meds
routine flu and pneumococcal shots

Question 6

drug treatment of PAH

Answer 6

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

Question 7

prostacyclin analogues (prostanoids) and receptor agonists

Answer 7

epoprostenol, treprostinil, remodulin, iloprost, selexipag
potent vasodilators and inhibitors of platelet aggregation
continuous IV at home

Question 8

epoprostenol dosing

Answer 8

continuous IV
2 ng/kg/min

Question 9

treprostinil dosing

Answer 9

Remodulin: continuous SC or IV infusion ng/kg/min

Question 10

prostacyclin analogues warnings

Answer 10

vasodilation (hypotension, flushing)
rebound PH
chronic iv infusions: infections

Question 11

prostacyclin analogues side effects

Answer 11

hypotension
flushing
jaw pain
infusion-site pain

Question 12

prostacyclin anagloues notes

Answer 12

parenteral agents: potent vasodilators; avoid interruptions and sudden, large dose reductions

epoprostenol: must protect from light during infusion

Flolan: reconstituted solution require ice packs

Question 13

endothelin receptor antagonists

Answer 13

bosentan, ambrisentan, macitentan

Question 14

endothelin receptor antagonists BBW

Answer 14

teratogenic: must have negative pregnancy test before starting and monthly during

Bosentan: hepatotoxicity

REMS program only

Question 15

endothelin receptor antagonists CI

Answer 15

pregnancy

Question 16

endothelin receptor antagonists warnings

Answer 16

hepatotoxicity
dec Hbg/Hct
fluid retention

Question 17

endothelin receptor antagonists side effects

Answer 17

headache

Question 18

endothelin receptor antagonist DI

Answer 18

Bosentan: dec effectiveness of hormonal contraceptive

Question 19

PDE-5 inhibitors

Answer 19

inc cGMP = pulmonary vasculature relaxation and vasodilation
sildenafil, tadalafil

Question 20

PDE-5 inhibitors CI

Answer 20

nitrates

Question 21

PDE-5 inhibitors warnings

Answer 21

hearing loss
vision loss (NAION)
hypotension
priapism

Question 22

PDE-t inhibitors side effects

Answer 22

headache

Question 23

PDE-5 inhibitor DI

Answer 23

do not use with nitreates = low BP; absolute CI

caution with alpha-blocker (or other antihypertensives) = hypotension

Question 24

soluble guanylate cyclase stimulator

Answer 24

guanylate cyclase is receptor for endogenous NO
Riociguat sensitizes sGC and directly stimulates = inc cGMP = relaxation of pulmonary artery smooth muscle cells

Question 25

Riociguat BBW

Answer 25

teratogenic (negative pregnancy test before and monthly during)
only through REMS

Question 26

riociguat CI

Answer 26

pregnancy
use of PDE-5i or nitreates

Question 27

riociguat warnings

Answer 27

hypotension

Question 28

riociguat side effects

Answer 28

headache

Question 29

riociguat DI

Answer 29

do not use with nitrate = dec BP

Question 30

pulmonary fibrosis

Answer 30

scarred and damaged lung tissue
presentation of exertional dyspnea