850-Pulmonary Arterial Hypertension (treatment algorithm)

Question 1

1. Treatment Naive PAH pts WHO FC II

OR

2. Treatment Naive PAH pts WHO FC III w/o evidence of rapid disease progression or poor prognosis
   - Is the pt willing or able to tolerate combination therapy?

YES

Answer 1

Combination therapy with ambrisentan & tadalafil

Question 2

1. Treatment Naive PAH pts WHO FC II

OR

2. Treatment Naive PAH pts WHO FC III w/o evidence of rapid disease progression or poor prognosis
   - Is the pt willing or able to tolerate combination therapy?

NO

Answer 2

Monotherapy with either

1. bosentan
2. macitentan
3. ambrisentan
4. riociguat
5. sildenafil
6. tadalafil

Question 3

1. PAH pts - WHO FC III w/ evidence of rapid disease progression or poor prognosis

OR

2. PAH pts w/ WHO FC IV
   - Is the patient willing & able to manage parenteral prostanoids?

YES

Answer 3

Continuous IV epoprostenol, IV treprostinil, or SC treprostinil

Question 4

PAH pts - WHO FC III w/ evidence of rapid disease progression or poor prognosis

-Is the patient willing & able to manage parenteral prostanoids?

NO

Answer 4

Consider addition of inhaled or oral prostanoid **

** No data available for the use of oral or inhaled prostanoids in patients in whom parenteral prostanoids are indicated, but patient is unable to comply. Thus, no specific recommendation for this population.

Question 5

PAH pts w/ WHO FC IV

-Is the patient willing & able to manage parenteral prostanoids?

NO

Answer 5

Inhaled prostanoid in combination w/ an oral PDE-5I & an oral ERA

Question 6

Pts w/ inadequate response to initial therapy

-For WHO FC III or IV PAH w/ unacceptable clinical status despite established PAH-specific Monotherapy

Answer 6

Addition of a second class of PAH therapy

Question 7

Pts w/ inadequate response to initial therapy

-For WHO FC III or IV PAH w/ unacceptable or deteriorating clinical status despite established PAH-specific therapy w/ two classes of PAH pharmacotherapy

Answer 7

Addition of a third class PAH therapy