Exam 6 - PAH Israel

Question 1

which is the gold standard for diagnosis of PAH?

a. echocardiogram
b. right heart catheterization
c. exercise testing
d. biomarkers

Answer 1

b. right heart catheterization

Question 2

what is the treatment for pt who is a positive responder to an acute vasoreactivity test?

Answer 2

CCB

Question 3

what two agents are used in an acute vasoreactivity test?

Answer 3

-inhaled nitric oxide
-IV epoprostenol

Question 4

what is the recommended combo treatment for WHO class II or class III pts?

Answer 4

ambrisentan + tadalafil

Question 5

what are the 3 preferred agents for monotherapy for WHO class II or class III pts without rapid progression?

Answer 5

-ERA (endothelin receptor antag)
-riociguat
-PDE-5 inhibitor

Question 6

how do PDE-5 inhibitors work for PAH?

Answer 6

they dec conversion of cGMP to GMP; inc levels of cGMP lead to pulmonary vasodilation

Question 7

true or false: PDE-5 inhibitors are considered first line in pts with FC II, FC III without rapid progression

Answer 7

true

Question 8

which is taken TID for PAH?

a. sildenafil (Revatio)
b. tadalafil (Adcirca)

Answer 8

a. sildenafil (Revatio)

(tadalafil is once daily)

Question 9

which ERA is given BID?

a. bosentan
b. ambrisentan
c. macitentan

Answer 9

a. bosentan

Question 10

which ERA is most likely to cause peripheral edema?

a. bosentan
b. ambrisentan
c. macitentan

Answer 10

b. ambrisentan

Question 11

which ERA causes the most LFT abnormalities?

a. bosentan
b. ambrisentan
c. macitentan

Answer 11

a. bosentan

Question 12

true or false: do not initiate ERA’s if LFT > 3 x ULN

Answer 12

true

Question 13

how long to see improvement after starting using an endothelin receptor antagonist? (range)

Answer 13

8-10 weeks

Question 14

what are the 3 preferred agents for WHO FC III (with rapid progression or poor prognosis) and WHO FC IV who are candidates for parenteral prostanoids?

Answer 14

-SC treprostinil
-IV treprostinil
-IV epoprostenol

Question 15

prostacyclin analogs inhibits __________ __________

Answer 15

platelet aggregation

Question 16

what are the 2 oral prostacyclin analogs?

Answer 16

-treprostinil (Orenitram)
-selexipag (Uptravi)

Question 17

which drug requires re-titration if therapy is interrupted for 3 days or more?

a. treprostinil (Orenitram)
b. selexipag (Uptravi)
c. tadalafil (Adcirca)
d. iloprost (Ventavis)

Answer 17

b. selexipag (Uptravi)

Question 18

two inhaled prostacyclin analogs

Answer 18

-iloprost (Ventavis)
-treprostinil (Tyvaso)

Question 19

what are the preferred agents for WHO FC III (with rapid progression or poor prognosis) and WHO FC IV who are NOT candidates for parenteral prostanoids?

Answer 19

inhaled or oral prostanoid (likely in combo w/ERA + PDE-5i)

Question 20

what drug class is standard for severe PH with right ventricular failure?

Answer 20

parenteral prostacyclins

(subQ treprostinil most common)

Question 21

half life of IV/subQ treprostinil

Answer 21

4 hours

Question 22

why do we prefer subQ prostacyclin vs IV?

Answer 22

IV has risk of central line infections

Question 23

ERAs and riociguat should be avoided in what pt population?

Answer 23

pregnancy

Question 24

which agents would be acceptable for initial monotherapy in a pt with WHO FC II PAH with significant comorbidities? (3 of them)

Answer 24

ERA, PDE-5, or riociguat