Who is the common demographic for pulmonary arterial HTN?
What is the definition of pulmonary arterial HTN?
sustained elevation of mean pulmonary arterial pressure >25mmHg at rest
What are the common s/s of pHTN?
DOE, fatigue, chest pain, tachycardia,
syncope/near-syncope, edema, cyanosis
What is the first gene linked to pHTN?
What common drug causes weight loss and pHTN?
What is the vasopressor test?
short acting vasodilator is administered
test is positive if PAP falls >10
CO is unchanged or increased
Some positive responders to the vasopressore test will achieve sustained functional improvement and prolonged suvival with what drug?
but will be deleterious in non-responders
What drugs do not help pHTN?
What is the MOA of prostanoids?
promotes vascular relaxation
What are the effects of prostanoids?
lowers pulmonary arterial resistance
decreases pulmonary arerial pressure
increases exercise tolerance
Epoprostenol has a (short or long) half life?
short half life
must be given by IV continuously and kept cold
What are some serious adverse effects of epoprostenol?
life-threatening if pump problems ensue
What are the pharmokokinetics of treprostinil?
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
What are the adverse effects for Treprostinil?
How is iloprost administered?
What are some adverse effects?
inhalation 6-9x per day
fainting from hyptension, jaw pain
Selexipag is a prostanoid that can be administered (oral or IV)?
common adverse effect?
is VERY expensive
Bosentan is an endothelin antagonist that blocks what?
blocks nonspecifically ETa and ETb receptors
What are some toxicities of bosentan?
accelerates warfarin metabolism and oral contraception
Ambrisentan is an endothelin antagonist that blocks what?
What are the main adverse reactions?
teratogenesis, no liver damage, does not interfere with warfarin, but still must use 2 forms of birth control (due to teratogenesis)
Macitentan is an endothelin antagonist that has what benefit?
18hr half life allowing for once/day dosing
Silidenafil blocks what?
what can it cause if combined with a-blockers or nitrates?
selectively blocks PDE 5
can cuase significant hypotension
What is the MOA of Riociguat?
sensistizes sGC to endogenous NO by stabilizing the NO-sGC
increases cGMP to increase vasodilation
If a pt with confirmed pHTN and has a positive vasoreactivty test, treat with what?
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?
Ambristentan and tadalafil
Macitentan, ambisentan, riocguat, sildenafil, or tadalafil
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?
Abrisentan an Tadalfil
Monotherapy with macitentan, ambisentan, riociguat, sildenafil, or tadalafil
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
IV epoprosteonol, IV treprostinil, or SC treprostinil
inhlaed or oral prostanoid
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
IV epoprostenol, IV treprostinil, or SC treprostinil
inhaled prostanoid + oral PDE5 inhibitor and oral ET antagonist
What is the most common pHTN drug combo?
Tadalafil + Ambrisentan
What are the prostanoids?
What are the PDE5 inhibitors