exam 6 lecture 4

Question 1

Where is pulmonary arterial HTN focused

Answer 1

It is focused on the pulmonary artery that is bringing de oxygenated blood away from the heart.

Question 2

Difference between PH and PAH

Answer 2

PH is mean pulmonary pressure (mPAP) > or = 20 mmHG at rest and is more common

PAH is a form of PH

PAH is rare

Question 3

causes of PAH

Answer 3

Unknown, genetic, drug and toxin exposure

Question 4

A majority of pts who have elevated PH is due to

Answer 4

HFrEF

Question 5

PAH epidemiology

Answer 5

4 X more common in women
mean 50 years old
15% mortality in 1 year
median survival 6 yrs

Question 6

signs and symptoms of PAH

Answer 6

86% SOB
27% fatugue
light headedness

Question 7

how to diagnose PAH

Answer 7

Right heart catheterization (gold standrad)
Echocardiogram
exercise testing

Question 8

which side of the heart has difficulty pumping against high pulmonary pressure

Answer 8

right

Question 9

WHO functional classes of PAH

Answer 9

Class I
Class II
Class III
Class IV

Question 10

Define class I in PAH

Answer 10

Symptom free when physically active or resting. No symptom

Question 11

Define Class II in PAH

Answer 11

Slight limitation of physical activity
comfortable at rest

Question 12

Define class III in PAH

Answer 12

Marked limitation in physical activity
less than ordinary activities cause symptoms
comfortable at rest

Question 13

Define Class IV in PAH

Answer 13

Significant sx at rest

Question 14

What medication to use if negative vasoreactivity test?

Answer 14

no CCB

Question 15

What medication to use if positive vasoreactivity test

Answer 15

CCB

Question 16

How to treat treatment naive FC I pts

Answer 16

Monitor progression
no drugs

Question 17

How to treat treatment naive FC II pts

Answer 17

If willing/able to tolerate combo therapy, ambiserten and tadalafil

if no- Monotherapy with bosentan, macisentan, ambisertan, sildenafil

Question 18

How to treat PAH patient with class III without rapid disease progression or poor prognisis

Answer 18

If pt is willing/able to tolerate Combo therapy- combo ambisentan and tadalafil

If no- Monotherapy with bosentan, macisentan, ambrisentan, riociguat, sildenafil or tadalafil

Question 19

How to treat PAH pts class III with evidence of rapid disease progression or poor prognosis

Answer 19

If patient is willling and able to manage parenteral prostanoids-

IV epoprostenol
IV treprostinil
SC treprostinil

If no- consider addition of inhaled or oral prostinoid combined with ERA and PDE-5

Question 20

How to treat PAH pts class IV

Answer 20

If patient is willling and able to manage parenteral prostanoids-

IV epoprostenol
IV treprostinil
SC treprostinil

If no- consider addition of inhaled or oral prostinoid combined with ERA and PDE-5

Question 21

IMPORTANT positive responders to acute vasoreactivity testing should be treated with

Answer 21

CCB

Question 22

When should we not use CCB for pAH

Answer 22

-ve testing to acute vasoreactivtiy
RV failure or CCB CI

Question 23

IMPORTANT Recommended CCB drugs

Answer 23

Nifedipine
Diltiazem
Amlodipine

NO VERAPAMIL

Question 24

summary of tx of PAH and different classes

Answer 24

Class 1- just monitor
Class 2- if tolerate combo therapy- Ambisentan + tadalafil

If no- Monotherapy ERA, PDE 5 I, Riociguat

Class 3 without rapid progression or poor prognosis- if tolerate combo therapy- Ambisentan + tadalafil

If no- Monotherapy ERA, PDE 5 I, Riociguat
(same as class II)

Class III with rapid progression- If candidate for parenteral prostanoids- SC treprostinil (preferred)
IV treprostinil
IV epoprostenol

If not Consider
inhaled or oral
prostanoid
(likely in combo
w/ERA + PDE-
5i)

Class IV same as III with poor prognosis

Question 25

Endothelin receptor antagonist drugs

Answer 25

Bosentan
Ambisentan
Macisentan

Question 26

prostacyclin pathway drugs

Answer 26

Epoprostenol
iloprost
Treprostenol

Question 27

How do PDE 5 drugs work

Answer 27

reduce conversion of cGMP to GMP. Causing vasodilation.

Question 28

IMPORTANT. PDE5 drug names and doses

Answer 28

SIldenafil (TID)
Tadalafil (QD)

Question 29

ADR for PDE5 drugs

Answer 29

Flushing, hypotension, headache

Question 30

Endothelin receptor MOA and drug name

Answer 30

Patients with PAH overexpress endothelin (vasoconstricor) Blocks endothelin causing less vasoconstriction

Ambisentan for ETa
bisentan/macisentan mixed

Question 31

ADR of Endothelin receptors, monitoring and CI?

Answer 31

Bosentan has highest risk of hepatic dysfunction
Ambrisentan has the most edema

monitor LFT monthly for bosentan

CI in pregnancy

Question 32

what is a soluble guanylate cyclase stimulator drug and what to know about it

Answer 32

Riociguat
CAN NOT be combined with tadalafil and sildenafil due to hypotension risk.

Question 33

IMPORTANT THING TO KNOW ABOUT prostacyclins

Answer 33

Have an effect on the aggregation of platelets

Question 34

IMPOTANT THING to know about treprostinil IV/SQ

Answer 34

IV infusion requires stable access, do not COINFUSE with anything else.

same with epoprostenol