PAH

Question 1

What treatment should be used based on AVT results?

Answer 1

• Positive: CCB
• Negative, RV failure, or CCB contra: do not use CCB

Question 2

What is the purpose of AVT?

Answer 2

Predicts response to CCB

Question 3

Which CCB should be avoided?

Answer 3

Verapamil (negative inotropic effects)

Question 4

WHO FC I
Require therapy?

Answer 4

• At risk, but symptom free when active or at rest
• Not immediate therapy, but consider CCB if responder

Question 5

WHO FC II
Require therapy?

Answer 5

• comfortable at rest, slight limitation of physical ability (ordinary activity causes symptoms)
• Yes; CCB if positive, targeted therapy if neg/contra CCB

Question 6

WHO FC III

Answer 6

comfortable at rest, marked limitation in physical activity

Question 7

WHO FC IV

Answer 7

symtoms at rest & activity

Question 8

What are examples of worsening symptoms

Answer 8

• dyspnea on exertion
• fatigue
• weakness

Question 9

What is targeted therapy for PAH (FC II, FC III w/o rapid progression or poor prognosis)

Answer 9

• Combo: ambrisentan & tadalafil
• Mono: ERA, riociguat, or PDE5i

Question 10

What is the treatment for FC III with rapid progression or poor prognosis

Answer 10

• if candidate for parenteral prostanoids, SC treprostinil, IV treprostinil, or IV epoprostenol
• if not, inhaled or oral prostanoid

Question 11

What is the treatment for FC IV?

Answer 11

• if candidate for parenteral prostanoids, SC treprostinil, IV treprostinil, or IV epoprostenol
• if not, inhaled prostanoid & ERA + PDE5i

Question 12

ERA

Answer 12

• Drugs: ambrisentan, macitentan, bosentan

Question 13

PDE5i

Answer 13

• Drugs: sildenafil, tadalafil

Question 14

Riociguat