exam 6 lecture 4 Flashcards
Where is pulmonary arterial HTN focused
It is focused on the pulmonary artery that is bringing de oxygenated blood away from the heart.
Difference between PH and PAH
PH is mean pulmonary pressure (mPAP) > or = 20 mmHG at rest and is more common
PAH is a form of PH
PAH is rare
causes of PAH
Unknown, genetic, drug and toxin exposure
A majority of pts who have elevated PH is due to
HFrEF
PAH epidemiology
4 X more common in women
mean 50 years old
15% mortality in 1 year
median survival 6 yrs
signs and symptoms of PAH
86% SOB
27% fatugue
light headedness
how to diagnose PAH
Right heart catheterization (gold standrad)
Echocardiogram
exercise testing
which side of the heart has difficulty pumping against high pulmonary pressure
right
WHO functional classes of PAH
Class I
Class II
Class III
Class IV
Define class I in PAH
Symptom free when physically active or resting. No symptom
Define Class II in PAH
Slight limitation of physical activity
comfortable at rest
Define class III in PAH
Marked limitation in physical activity
less than ordinary activities cause symptoms
comfortable at rest
Define Class IV in PAH
Significant sx at rest
What medication to use if negative vasoreactivity test?
no CCB
What medication to use if positive vasoreactivity test
CCB
How to treat treatment naive FC I pts
Monitor progression
no drugs
How to treat treatment naive FC II pts
If willing/able to tolerate combo therapy, ambiserten and tadalafil
if no- Monotherapy with bosentan, macisentan, ambisertan, sildenafil
How to treat PAH patient with class III without rapid disease progression or poor prognisis
If pt is willing/able to tolerate Combo therapy- combo ambisentan and tadalafil
If no- Monotherapy with bosentan, macisentan, ambrisentan, riociguat, sildenafil or tadalafil
How to treat PAH pts class III with evidence of rapid disease progression or poor prognosis
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
How to treat PAH pts class IV
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
IMPORTANT positive responders to acute vasoreactivity testing should be treated with
CCB
When should we not use CCB for pAH
-ve testing to acute vasoreactivtiy
RV failure or CCB CI
IMPORTANT Recommended CCB drugs
Nifedipine
Diltiazem
Amlodipine
NO VERAPAMIL
summary of tx of PAH and different classes
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
Endothelin receptor antagonist drugs
Bosentan
Ambisentan
Macisentan
prostacyclin pathway drugs
Epoprostenol
iloprost
Treprostenol
How do PDE 5 drugs work
reduce conversion of cGMP to GMP. Causing vasodilation.
IMPORTANT. PDE5 drug names and doses
SIldenafil (TID)
Tadalafil (QD)
ADR for PDE5 drugs
Flushing, hypotension, headache
Endothelin receptor MOA and drug name
Patients with PAH overexpress endothelin (vasoconstricor) Blocks endothelin causing less vasoconstriction
Ambisentan for ETa
bisentan/macisentan mixed
ADR of Endothelin receptors, monitoring and CI?
Bosentan has highest risk of hepatic dysfunction
Ambrisentan has the most edema
monitor LFT monthly for bosentan
CI in pregnancy
what is a soluble guanylate cyclase stimulator drug and what to know about it
Riociguat
CAN NOT be combined with tadalafil and sildenafil due to hypotension risk.
IMPORTANT THING TO KNOW ABOUT prostacyclins
Have an effect on the aggregation of platelets
IMPOTANT THING to know about treprostinil IV/SQ
IV infusion requires stable access, do not COINFUSE with anything else.
same with epoprostenol
