PAH

Question 1

PH defined by:

Answer 1

mPAP > 20 at rest via rest heart catheterization

PVR > 2 wood units

Question 2

Acute Vasodilator Testing

Answer 2

-Responder if mPAP decreases by 10, to value of < 40 without decrease in CO
-Indicates CCB

*Not performed in right HF or instability

Question 3

CCB

Answer 3

-Must be responder to vasodilator testing

Agents: nifedipine, diltiazem, amlodipine
-Avoid verapamil

AE: edema, ging hyperplasia, HR

Question 4

ERAs
-Bosentan, Ambrisentan, Macitentan
(TANS)

Answer 4

Warnings
-Teratogenic
-REMs (monitor pregnancy)

Bosentan
-Also monitor LFTs
Ambri
-Edema
Maciten
-Congestion, UTI

BL AE CUM

Question 5

Require REMS

Answer 5

-ERAs
-Riociguat

Question 6

PDE5i (Sildenafil, Tadalafil)

Answer 6

Warnings
-Hypotension
-PDE5is are CI with nitrates or guanylate cyclase

Sildenafil = 20 mg TID
-AE: HA, flush, dysp, nose bleed, visual impairment (FDB HIV)

Tadalafil = 20-40 QD

DDI: CYPs

Question 7

Riociguat (sGC)

Answer 7

0.5-1 mg TID
-Increase by 0.5 every 2 weeks TID
-Target 2.5 mg TID

AE: dyspepsia, gastritis, hypotension

CI: nitrates, PDE5is

DDI: CYP

No smoking

Question 8

Prostacyclins (Epoprostenol, Treprostinil, Iloprost, Selexipag)

Answer 8

Warnings
-Serious AEs
-Central line infections
-Risk of abrupt d/c

Question 9

Epoprostenol

Answer 9

GOLD standard for high risk pts

2 ng/kg/min titrate to 25-45

AE: JIMI FRND
-HA, ND, rash, flushing
-Jaw pain, musco pain
-Infections
-Interrupt infusion = life threatening

Question 10

Treprostinil

Answer 10

Similar AE to epo
-HA, NVD
-Infusion site pain or reaction

Oral
0.125 mg TID or 0.25 BID
(max 120 mg TID)

Question 11

Inhaled Prostacyclins

Answer 11

Iloprost
6-9x per day

Treprostinil neb
3-9 breaths 4x per day

Treprostinil dry power
1 breath 4x per day

Question 12

Selexipag

Answer 12

Avoid in severe hepatic failure
(moderate = 200 mcg starting)

With or without food, do not crush or chew

AE: HA, ND, MJ pain

DONT GIVE WITH CLOPIDOGREL

Question 13

Guideline Algorithm

Answer 13

WITH cardiopulmonary comorbidities
-1. PDE5i or ERA

WITHOUT
-1. Low/Int risk = ERA + PDE5i
-2. High (20%+) = ERA + PDE5i + IV/SC PCA
-3. Add PRA or switch from PDE5i to RIO (If high risk, add IV/SC PCA or eval for lung transplant)

Question 14

Supportive Tx

Answer 14

-Diet, less sodium
-Exercise
-Vaccinations
-Anticoag if not CI
-Oxygen
-Caution in high altitude
-Diuretics

Question 15

Drugs that induce PAH

Answer 15

Dasatinib, pheny/pheno, methamphetamine