Pulmonary HTN Drugs Flashcards

1
Q

When do you use a CCB to treat PH?

A

if positive vasopressor test

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2
Q

What is the definition of PAH?

A

sustained elevation of mPAP to 25 mmHg or greater

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3
Q

Who is the typical pt w/ PAH?

A

young mother

more common in females, can occur at any age

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4
Q

What is the significance of BMPR2?

A

first gene linked to PAH, but seen in <25% of idiopathic PAH

gene product is supposed to be a brake to vasoconstriction system

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5
Q

Generally, what is the cellular theory of development of PAH?

A

messed up K+ channels –> more calcium in cell –> contraction, proliferation, and apoptosis

serotonin and endothelin I further increase calcium to make this worse

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6
Q

What can you see on CXR in PAH?

A

peripheral hypovascularity in lungs

prominent central pulmonary A

RV enlargement

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7
Q

What do you see on ECG in PAH?

A

right axis deviation of QRS complex

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8
Q

What is the vasopressor test for PAH?

A

short-acting vasodilator given –> positive if PaP falls 10 or more, mean pulm A pressure drops 40 or more, CO is unchanged or increased

+ test can signal you to prescribe non-dyh CCBs

If negative –> don’t use CCBs

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9
Q

What are the extra txs besides specific PAH meds?

A

anticoagulants

diuretics

Oxygen therapy

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10
Q

How are prostacyclin analogs given?

A

continuous IV, subcutaneous infusion or intermittent nebulizer

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11
Q

What are the 4 basic groups of PAH meds?

A

prostacyclin analogs

endothelin receptor antagonists

PDE-5 inhibitors

soluble guanylyl cyclase stimulants

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12
Q

How is epoprostenol given?

Adverse effects?

A

continuous IV that has to be kept cold (short half-life)

sepsis due to chronic catheter

nausea and vomiting, HA, flushing, jaw pain

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13
Q

what are the 3 benefits prostacyclin analogs have?

A

promotes vascular relaxation

suppresses growth of vasc smooth muscle cells

inhibits platelet aggregation

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14
Q

How is treporstinil given?

Side effects?

A

subcut infusion, but painful –> now often diluted and admin w/ pump IV (like epoprostenol)

longer half life and no refrigeration

S/e similar to that of epoprostenol (sepsis, general systemic sx)

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15
Q

How is iloprost given?

Side effects?

A

inhalation 6-9 times/day

severe: fainting due to hypotension, cough, HA, flushing, jaw pain

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16
Q

How is selexipag given?

A

admin orally BID = easier than other prostanoids, but super expensive!

17
Q

what is the suffix for endothelin antagonists?

A

-entan

(bosentan, ambrisentan, and macitentan)

18
Q

What is the word root for prostanoids and what are those drugs?

A

-prost

epoprostenol, treprostinil, iliprost, selexipag (exception to word rule)

19
Q

What is the half life of sildenafil?

A

~4 hrs

tadalafil has longer

20
Q

What is riociguat’s MOA?

A

soluble guanylate cyclase sensitizer –> directly stimulates independent of NO –> vasodilation

increases generation of cGMP –> incr vasodilation

21
Q

What is the effect of all PAH drugs except prostanoids?

A

improves exercise tolerance and slows sx progression

22
Q

What is the MOA of bosentan?

adverse effects?

A

non-spec block of ETa and ETb endothelin receptors

hepatotoxicity, teratogenesis

accelerates metabolism of warfarin and oral contraceptives (must use 2 forms of birth control)

23
Q

How are endothelin antagonists given?

A

orally

24
Q

What is the MOA of ambrisentan?

Adverse effects?

A

selectively blocks Eta receptors that cause vasoconstriction and promote cell proliferation

teratogensis, *but doesn’t damage liver (vs bosentan)

*doesn’t accelerate metabolism of warfaran and Oral contraceptives vs bosentan

but super expensive!

25
Q

What distinguishes macitentan from other endothelin antagonists?

A

18 hr half life that permits pid dosing

26
Q

How is riociguat given?

What should it not be given with?

A

oral - half life ~ 12 hrs

don’t give w/ NO donors or PDE-5 inh

teratogen potential

27
Q

What is a worry of all endothelin antagonists?

A

teratogenesis

28
Q

What pulm HTN drug has caused sudden hearing loss and visual disturbances in some people?

A

PDE-5 inhibitors

29
Q

How do you tx pts w/ FC II or III (w/o evidence of rapid dz progression) Pulm HTN ?

A

either combo w/ ambrisentan and tadalafil

or monotherapy w/ either macitentan, ambisentan, riociguat, sildenafil or tadalafil

30
Q

How do you tx pt w/ FC III Pulm htn w/ evidence of rapid dz progression?

A

continuous IV epoprostenol, IV treprostinil or SC treprostinil

or consider addition of inhaled or oral prostanoid

31
Q

How do you tx PAH pt w/ FC IV?

A

continue IV epoprostenol, IV treprostinil, or SC treprostinil

or: inhaled prostanoid + oral PDE-5 inh and oral ET-antagonist