PAH Drugs

Question 1

Sprycel

Answer 1

Dasatinib
(Causes PAH)

Question 2

Proglycerin

Answer 2

Diazoxide
(Causes PAH)

Question 3

Fintepla

Answer 3

Fenfluramine
(Causes PAH)

Question 4

Flolan, Veletri

Answer 4

Epoprostenol
(Prostacyclin analogue and receptor agonist)

Question 5

Remodulin (continous SC or IV)

Tyvaso (inhalation)

Orenitram (oral, ER tablet)

Answer 5

Treprostinil

(Prostacyclin analogue and receptor agonist)

Question 6

Ventavis (inhalation)

Answer 6

Iloprost

(Prostacyclin analogue and receptor agonist)

Question 7

Uptravi (tablet)

Answer 7

Selexipag

(Prostacyclin analogue and receptor agonist)

Question 8

HF contraindications

Answer 8

(Prostacyclin analogue and receptor agonist)

Question 9

Contraindications child-pugh class C

Answer 9

Treprostinil

Question 10

Vasodilatory side effect
Sepsis/Bactermia
Jaw pain
Rebound PH
Increased bleeding risk

Answer 10

Prostacyclin analogue and receptor agonist

Question 11

Short half life and requires back up pump/drugs

Answer 11

Epoprostenol
Treprostinil

Question 12

Requires protection from light

Answer 12

Epoprostenol

Question 13

Requires ice pack for stability after reconstitution

Answer 13

Flolan (Epoprostenol)

Question 14

Level affected by 2C8 inhibitors and inducers

Answer 14

Treprostinil
Selexipag

Question 15

Tracleer

Answer 15

Bosentan
(endothelin receptor antagonist)

Question 16

Letairis

Answer 16

Ambrisetan
(endothelin receptor antagonist)

Question 17

Opsumit

Answer 17

Macitentan

(endothelin receptor antagonist)

Question 18

< 40kg: 62.5 BID
>= 40kg: 62.5 BID x 4weeks —> 125mg BID

Answer 18

Bosentan

Question 19

5mg daily may increase to 10mg after 4 weeks

Answer 19

Ambrisetan

Question 20

10 mg daily

Answer 20

Macitetan

Question 21

Requires REMS
Teratogenic
Hepatotoxicity
Reduces H&H

Answer 21

(endothelin receptor antagonist)

Question 22

Hypersensitivity reaction
3A4 and 2C9 substrate and inhibitors
Reduces contraceptive effectiveness
Avoid with cyclosporine and glyburide
Greater risk of hepatotoxicity
Approved for 3 yrs and older

Answer 22

Bosentan

Question 23

Avoid in idiopathic pulmonary fibrosis
3A4, 2C19 and P-gp substrate

5mg daily if with cyclosporine

Answer 23

Ambrisentan

Question 24

3A4 and 2C19 substrate

Answer 24

Macitetan

Question 25

Reviatio (PAH) Viagra (erectile dysfunction)

Answer 25

Sildenafil (PDE-5 inhibitors)

Question 26

Adcirca, Alvq (PAH) Cialis (erectile dysfunction)

Answer 26

Tadalafil

Question 27

Tablet, oral suspension, IV

Answer 27

Revatio

Question 28

Avoid renal and hepatics impairments Avoid with alpha-blockers

Answer 28

Adcirca, Alvq

Question 29

Avoid with protease inhibitors

Answer 29

Reviatio

Question 30

Avoid with nitrates and alcohol Caution with alpha blocker Major 3A4 substrate

Answer 30

(PDE-5 inhibitors)

Question 31

Vasodilation effects Praipism Epistaxis Eye and Ear loss

Answer 31

(PDE-5 inhibitors)

Question 32

Adempas

Answer 32

Riociguat (Soluble guanylate cyclase stimulator)

Question 33

Major 3A4, 2C8, & P-gp substrate

Answer 33

Riociguat

Question 34

Teratogenic Requires REMS Avoid with PDE5i and nitrates Separate from antacids > 1 hour Smoking decreases clearance

Answer 34

Riociguat

Question 35

Separate from sildenafil by 24hrs and tadalafil by 48 hrs

Answer 35

Riociguat

Question 36

Max dose 2.5 mg if SBP > 95 mmHg

Answer 36

Riociguat

Question 37

Approved for PAH and CTEPH

Answer 37

Riociguat

Question 38

Amiodarone/Dronedarone Bleomycin Busulfan Carmustine/Lomustine Nitrofurantoin Sulfasalazine

Answer 38

Causes pulmonary fibrosis

Question 39

Esbriet

Answer 39

Pirfenidone (Approved for pulmonary fibrosis)

Question 40

Ofev

Answer 40

Nintedanib (Approved for pulmonary fibrosis)