Drugs for Pulmonary HTN

Question 1

Drugs in prostanoid class

Answer 1

• epoprostenol
• treprostinil
• iloprost
• selexipag

Question 2

MOA prostanoids

Answer 2

mimics endogenous prostacyclin to cause vascular relaxation, decrease growth of vascular smooth muscle, and inhibit platelet aggregation

Question 3

describe epoprostenol

Answer 3

• prostanoid
• short 1/2 life (6 min)
• need to give in continuous IV that keeps the bag cold
• risk of sepsis due to indwelling catheter

Question 4

describe treprostinil

Answer 4

• prostanoid
• given subcutaneously
• longer 1/2 life and no need for refrigeration (like epoprostenol)

Question 5

describe iloprost

Answer 5

• prostanoid

- given by inhalation 6-9x per day

Question 6

describe selexipag

Answer 6

• prostanoid
• given orally; BID
• EXPENSIVE $225- $350 each dose

Question 7

What are the endothelin antagonists?

Answer 7

• Bosentan
• Ambrisentan
• Macitentan

Question 8

describe Bosetan

Answer 8

• endothelin antagonist
• blocks ETa/ETb receptor
• improves exercise tolerance

Question 9

side effects Bosetan

Answer 9

• hepatotoxicity
• teratogenesis
• BAD w/ oral contraceptive
• accelerates warfarin metabolism

Question 10

describe Ambrisentan

Answer 10

• endothelin antagonist
• blocks ETa receptor
• teratogen
• no change in warfarin metabolism
• still need to use 2 forms of contraceptives

Question 11

describe Macitentan

Answer 11

• endothelin antagonist
• non selective agent
• take 1x per day (long 1/2 life)
• teratogen

Question 12

What is a concern for all endothelin receptor blockers

Answer 12

teratogenesis

Question 13

MOA PDE 5 inhibitors

Answer 13

block conversion cGMP –> 5’ GMP to promote vascular relaxation

Question 14

PDE5 inhibitors used for pulmonary HTN

Answer 14

• Sildenafil (viagra)

- Tadalafil (cialis)

Question 15

benefits of using PDE5 inhibitors in pulmonary HTN

Answer 15

improve exercise tolerance and slow Sx progression

Question 16

MOA guanylate cyclase sensitizer

Answer 16

sensitizes soluble guanylate cyclase (sGC) to endogenous nitric oxide by stabilizing NO-sGC binging
(increased cGMP means increased vasodilation)

Question 17

describe Riociguat

Answer 17

• guanylate cyclase sensitizer
• may cause fetal harm
• may interact with drugs at CYP450

Question 18

definition pulmonary HTN

Answer 18

MAP >25 mmHg at rest

Question 19

Who is pulmonary HTN common in?

Answer 19

young mothers, but more common in females of any age

Question 20

Pulmonary artery changes in PAH

Answer 20

1) vasoconstriction
2) inflammation
3) localized thrombus formation
4) obstructive remodeling of vessel wall

Question 21

When are CCBs used

Answer 21

if vasopressor test is positive

Question 22

what CCBs are used with positive vasopressor test

Answer 22

• nifedipine
• diltiazem
• amlodipine

Question 23

describe vaspressor test

Answer 23

• short acting vasodilator administered
• test is positive if PAP falls more than 10 mmHg/MAP is less than 40 mmHG
• give CCBs in addition if positive

Question 24

Symptoms pulmonary HTN

Answer 24

• dyspnea on exertion
• syncope
• swelling in legs/ankles
• cyanotic lips/skin

Question 25

Risk factors pulmonary HTN

Answer 25

- family history - BMPR2 gene - HIV infection - portal HTN - fen/phen use

Question 26

Symptomatic profile of WHO functional class I of pulmonary HTN

Answer 26

patients with pulmonary HTN but without resulting limitation of physical activity

Question 27

Symptomatic profile of WHO functional class II of pulmonary HTN

Answer 27

patients with pulmonary HTN resulting in slight limitation of physical activity. Comfortable at rest. Ordinary physical activity causes dyspnea/fatigue

Question 28

Symptomatic profile of WHO functional class III of pulmonary HTN

Answer 28

patients with pulmonary HTN resulting in marked limitation of physical activity. Comfortable at rest. Less than ordinary physical activity causes dyspnea/fatigue

Question 29

Symptomatic profile of WHO functional class IV of pulmonary HTN

Answer 29

Patients with pulmonary HTN with inability to carry out any physical activity without symptoms. Manifest signs of right heart failure

Question 30

Which class of drugs has caused sudden hearing loss and/or visual disturbances (e.g., lack of color discrimination) when used by some patients?

Answer 30

PDE 5 inhibitors

Question 31

What drug combo is typically used for patients with WHO FC II pulmonary HTN?

Answer 31

ambrisentan and tadalafil

Question 32

At what point are prostanoids indicated for treatment-naive patients requiring therapy for pulmonary hypertension?

Answer 32

WHO FC III, evidence of rapid disease progression