Drugs for Pulmonary HTN

Question 1

Who is the common demographic for pulmonary arterial HTN?

Answer 1

young women

Question 2

What is the definition of pulmonary arterial HTN?

Answer 2

sustained elevation of mean pulmonary arterial pressure >25mmHg at rest

Question 3

What are the common s/s of pHTN?

Answer 3

DOE, fatigue, chest pain, tachycardia,

syncope/near-syncope, edema, cyanosis

Question 4

What is the first gene linked to pHTN?

Answer 4

BMPR2

Question 5

What common drug causes weight loss and pHTN?

Answer 5

fen/phen

Question 6

What is the vasopressor test?

Answer 6

short acting vasodilator is administered

test is positive if PAP falls >10

MPAP <40

CO is unchanged or increased

Question 7

Some positive responders to the vasopressore test will achieve sustained functional improvement and prolonged suvival with what drug?

Answer 7

CCB

but will be deleterious in non-responders

Question 8

What drugs do not help pHTN?

Answer 8

anticoagulants

diuretics

O2 therapy

Question 9

What is the MOA of prostanoids?

Answer 9

promotes vascular relaxation

increases cAMP

Question 10

What are the effects of prostanoids?

Answer 10

lowers pulmonary arterial resistance

decreases pulmonary arerial pressure

increases exercise tolerance

improves survival

Question 11

Epoprostenol has a (short or long) half life?

Answer 11

short half life

must be given by IV continuously and kept cold

Question 12

What are some serious adverse effects of epoprostenol?

Answer 12

sepsis

life-threatening if pump problems ensue

Question 13

What are the pharmokokinetics of treprostinil?

Answer 13

given SUBQ but caused too much pain, so given with IV pump

longer half life, no refrigeration

can do QID inhalation

extended release oral form avaialble

Question 14

What are the adverse effects for Treprostinil?

Answer 14

sepsis

jaw pain

cough/throat irritation

Question 15

How is iloprost administered?

What are some adverse effects?

Answer 15

inhalation 6-9x per day

fainting from hyptension, jaw pain

Question 16

Selexipag is a prostanoid that can be administered (oral or IV)?

common adverse effect?

Answer 16

Orally, BID

is VERY expensive

Jaw pain

Question 17

Bosentan is an endothelin antagonist that blocks what?

Answer 17

blocks nonspecifically ETa and ETb receptors

Question 18

What are some toxicities of bosentan?

Answer 18

hepatotoxicity

teratogenesis

accelerates warfarin metabolism and oral contraception

Question 19

Ambrisentan is an endothelin antagonist that blocks what?

What are the main adverse reactions?

Answer 19

Eta selectively

teratogenesis, no liver damage, does not interfere with warfarin, but still must use 2 forms of birth control (due to teratogenesis)

Question 20

Macitentan is an endothelin antagonist that has what benefit?

Answer 20

18hr half life allowing for once/day dosing

Question 21

Silidenafil blocks what?

what can it cause if combined with a-blockers or nitrates?

Answer 21

selectively blocks PDE 5

can cuase significant hypotension

Question 22

What is the MOA of Riociguat?

Answer 22

sensistizes sGC to endogenous NO by stabilizing the NO-sGC

increases cGMP to increase vasodilation

Question 23

If a pt with confirmed pHTN and has a positive vasoreactivty test, treat with what?

Answer 23

CCB

Question 24

In patient with naive PHTN with WHO FC II and patient is able to tolerate combination therapy, treat with what?

If they are unable to tolerate combo therapy, treat with what?

Answer 24

Ambristentan and tadalafil

Macitentan, ambisentan, riocguat, sildenafil, or tadalafil

Question 25

In pt with naive PAH with WHO FC III w/o evidence of rapid dz progression who is able to take combo theraoy, treat with what? If unable to take combo therapy?

Answer 25

Abrisentan an Tadalfil Monotherapy with macitentan, ambisentan, riociguat, sildenafil, or tadalafil

Question 26

In pt with naive PHTN with WHO FC III with evidence of rapid dz progression and able to take parenteral prostanoids, treat with IF unable to take parental prostanoids, take

Answer 26

IV epoprosteonol, IV treprostinil, or SC treprostinil inhlaed or oral prostanoid

Question 27

In pt with pHTN with WHO FC IV and able to take parenteral prostanoids, treat with waht? If unable to take parentarel prostenoids, tx with

Answer 27

IV epoprostenol, IV treprostinil, or SC treprostinil inhaled prostanoid + oral PDE5 inhibitor and oral ET antagonist

Question 28

What is the most common pHTN drug combo?

Answer 28

Tadalafil + Ambrisentan

Question 29

What are the prostanoids?

Answer 29

Epoprostenol treprostinil iloprost selexipag

Question 30

What are the PDE5 inhibitors

Answer 30

sildenafil tadalafil

Question 31

what are the endothelin antagonists?

Answer 31

bosentan ambrisentan macicentan

Question 32

What is the guanylate cyclase sensitizer

Answer 32

riociguat