Drugs for Pulmonary HTN

Question 1

list 4 prostanoids

Answer 1

• epoprostenol
• treprostinil
• iloprost
• selexipag

Question 2

list 3 endothelin antagonists

Answer 2

• bosentan
• ambrisentan
• macicentan

Question 3

list the 2 PDE 5 Inhibitors

Answer 3

• sildenafil

- tadalafil

Question 4

list the guanylate cyclase sensitizer

Answer 4

riociguat

Question 5

describe WHO functional class I

Answer 5

• pts with pulm HTN but w/o resulting limitation of physical activity
• ordinary activity does not cause dyspnea or fatigue, CP, or near syncope

Question 6

describe WHO functional class II

Answer 6

• pts with pulm HTN w/ slight limitation of physical activity
• comfortable at rest
• ordinary physical activity causes undue dyspnea or fatigue, CP, or near syncope

Question 7

describe WHO functional class III

Answer 7

• pts w/ pulm HTN w/ marked limitation of physical activity
• comfortable at rest
• less than ordinary activity causes undue dyspnea or fatigue, CP, or near syncope

Question 8

describe WHO functional class IV

Answer 8

• pts w/ pulm HTN w/ inability to carry out any physical activity w/o sx
• manifest signs of right HF
• dyspnea and fatigue may be present at rest

Question 9

MOA epoprostenol

Answer 9

prostanoid
- mimic actions of endogenous prostacyclin –> promotes vascular relaxation, suppresses growth of vascular smooth m. cells, inhibits platelet aggregation

Question 10

effects epoprostenol

Answer 10

• lower pulm art. pressure
• decreases pulmonary arterial resistance
• increases exercise tolerance
• improves short term survival

Question 11

half life and administration of epoprostenol

Answer 11

• short have life (6 mins)

- continuous IV infusion w/ pump that keeps it cold

Question 12

toxicities of epoprostenol

Answer 12

• sepsis due to chronic catheter
• nausea/vomiting
• HA
• flushing
• jaw pain

Question 13

MOA treprostinil

Answer 13

prostanoid
- mimic actions of endogenous prostacyclin –> promotes vascular relaxation, suppresses growth of vascular smooth m. cells, inhibits platelet aggregation

Question 14

effects treprostinil

Answer 14

• lower pulm art. pressure
• decreases pulmonary arterial resistance
• increases exercise tolerance
• improves short term survival

Question 15

half life and administration treprostinil

Answer 15

• longer half life than epoprostenol at 4 hrs

- diluted and administered with pump IV, doesn’t require refrigeration

Question 16

toxicities treprostinil

Answer 16

• sepsis due to chronic catheter
• jaw pain
• diarrhea
• edema
• nausea

Question 17

MOA iloprost

Answer 17

prostanoid
- mimic actions of endogenous prostacyclin –> promotes vascular relaxation, suppresses growth of vascular smooth m. cells, inhibits platelet aggregation

Question 18

effects iloprost

Answer 18

• lower pulm art. pressure
• decreases pulmonary arterial resistance
• increases exercise tolerance
• improves short term survival

Question 19

administration of iloprost

Answer 19

inhalation 6-9 times per day

Question 20

toxicities iloprost

Answer 20

• fainting due to hypotension
• cough
• HA
• flushing
• jaw pain

Question 21

MOA selexipag

Answer 21

prostanoid
- mimic actions of endogenous prostacyclin –> promotes vascular relaxation, suppresses growth of vascular smooth m. cells, inhibits platelet aggregation

Question 22

effects selexipag

Answer 22

• lower pulm art. pressure
• decreases pulmonary arterial resistance
• increases exercise tolerance
• improves short term survival

Question 23

administration of selexipag

Answer 23

orally BID

Question 24

toxicities selexipag

Answer 24

• HA
• flushing
• diarrhea/nausea/vom
• jaw, limb, muscle pain
• skin rash

Question 25

MOA bosentan

Answer 25

endothelin antagonist | - nonspecifically blocks ETA and ETB endothelin receptors

Question 26

effects bosentan

Answer 26

improves exercise tolerance and slows sx progression

Question 27

administration and metabolism of bosentan

Answer 27

oral drug metabolized by liver, highly protein bound

Question 28

toxicities bosentan

Answer 28

- hepatotoxicity - teratogenesis - HA, flushing, peripheral edema, nasal congestion, anemia

Question 29

drug interactions bosentan

Answer 29

accelerate metabolism of warfarin and oral contraceptives

Question 30

MOA ambrisentan

Answer 30

endothelin antagonist | - selectively blocks ETA receptors (that normally cause vasoconstriction and promote cell proliferation)

Question 31

effects ambrisentan

Answer 31

improves exercise tolerance and slows sx progression

Question 32

administration ambrisentan

Answer 32

oral

Question 33

toxicities ambrisentan

Answer 33

- teratogenesis - NO hepatotoxicity - peripheral edema, nasal congestion, anemia

Question 34

drug interactions ambrisentan

Answer 34

does NOT accelerate metabolism of warfarin and oral contraceptives

Question 35

MOA macitentan

Answer 35

endothelin antagonist | - nonselective agent

Question 36

administration and half life macitentan

Answer 36

18hr half life once a day dosing

Question 37

toxicities of ambrisentan

Answer 37

CYP450 effects similar to bosentan

Question 38

MOA sildenafil

Answer 38

blocks phosphodiesterase type V that breaks down cGMP

Question 39

effects sildenafil

Answer 39

improves exercise tolerance and slows sx progression

Question 40

administration and half life sildenafil

Answer 40

oral half life 4 hours

Question 41

toxicities of sildenafil

Answer 41

well tolerated - flushing - HA - dyspepsia - can cause visual disturbances

Question 42

MOA riociguat

Answer 42

guanylate cyclase sensitizer - sensitizes soluble guanylate cyclase to endogenous NO by stabilizing the NO-sGC binding - directly stimulates sGC indepdendent of NO - increased generation of cGMP --> increased vasodilation

Question 43

effects riociguat

Answer 43

improves exercise tolerance and slows sx progression

Question 44

administration and half life of riociguat

Answer 44

oral drug 12 hour half life

Question 45

toxicities of tiociguat

Answer 45

- hypotension, HA, dizziness, dyspepsia | - may cause fetal harm