PAH 2019 CHEST guideline

Question 1

what is the first step of 2019 CHEST guideline for PAH

Answer 1

see if patient has positive AVT

Question 1

what is 1st line PAH tx for +AVT pts

Answer 1

CCBs:
- nifedipine 120-240 mg daily
- diltiazem 240-720 mg daily
- amlodipine 20 mg daily

Question 2

what is tx for PAH functional class 1

Answer 2

monitor disease progression

Question 3

what is tx for PAH functional class 2

Answer 3

If pt is willing to do combo therapy:
- tadalafil + ambrisentan
If pt cannot or will not do combo therapy, do monotherapy of:
- PDE-5i, riociguat, oral ambrisentan, macitentan, bosentan

Question 4

what is tx for PAH functional class 3 without rapid progression

Answer 4

If pt is willing to do combo therapy:
- tadalafil + ambrisentan
If pt cannot or will not do combo therapy, do monotherapy of:
- PDE-5i, riociguat, oral ambrisentan, macitentan, bosentan

Question 5

what is tx for PAH functional class 3 with rapid progression

Answer 5

If pt is willing and can do parenteral prostanoids:
- subQ treprostinil, IV treprostinil or epoprostenil
If pt cannot take parenteral prostanoids:
- oral or inhaled prostanoids

Question 6

what is tx for PAH functional class 4 pts that can tolerate parenteral prostanoids

Answer 6

• subQ treprostinil
• IV treprostinil or epoprostinil

Question 7

what is tx for PAH functional class 4 pts that cannot tolerate parenteral prostanoids

Answer 7

oral or inhaled prostanoids + PDE-5i + ERA

Question 8

what to do if pt still has inadequate response to therapy

Answer 8

• for functional class 3 and 4 patients that have taken one therapy, add a second PAH therapy
• for functional class 3 and 4 patients have taken two therapies, add a third PAH therapy

Question 9

what to do if patient is still not responsive to maximally dosed PAH therapies

Answer 9

• consider lung transplantation if a candidate
• if not a lung transplantation candidate, start palliative care