Pulmonary Arterial Hypertension

Question 1

What is the most common PH group

Answer 1

Group 1: Idiopathic

Question 2

Drugs that cause PH

Answer 2

Cocaine
Fenfluramine
Meth/Amph
SSRI during pregnancy
Weight loss drugs (stimulants)

Question 3

Patho of PH

Answer 3

↑ in END-1 and TXA2
↓ in prostacyclin

Question 4

Most common cause of death from PH

Answer 4

HF → right ventricle becoming enlarged and failure develops

Question 5

Sx of PH

Answer 5

Fatigue, dyspepsia

Question 6

Non drug treatment for PH

Answer 6

Sodium-restriction <2.4 g/day
Avoid NSAIDs
Flu and Pneumococcal shot
O2

Question 7

How to diagnose PH

Answer 7

Right heart catherization
Vasoreactivity test

Question 8

What is a responder of vasoreactive test?

Answer 8

mPAP falls at least 10 mmHg to absolute value less than 40 mmHg

Question 9

Drugs for vasoreactivity positive?

Answer 9

Long-acting nifedipine, diltiazem, amlodipine

Verapamil is not recommended due to more pronounced negative inotropic effects compared to diltiazem

Question 10

Tx for non-responders

Answer 10

Prostacyclin analogs and receptor agonists
Endothelian receptor antagonists (ERAs)
PDE-5 (Adcirca)
soluble guanylate cyclase (sGC) stimulator

Question 11

Supportive therapies for PH

Answer 11

Loop diuretics: volume overload
Digoxin: improve cardiac output or control heart rate
Warfarin: For increased prothrombin states

Question 12

MOA of prostacyclin analogues

Answer 12

Potent vasodilators inhibiting platelet aggregation

Question 13

Examples of PCA?

Answer 13

Epoprostenol and treprostinil: continuous infusion

Question 14

Epoprostenol

Answer 14

Flolan

Question 15

Treprostinil

Answer 15

Remodulin

Question 16

Starting dose of Flolan

Answer 16

2 ncg/kg/min

Question 17

Remodulin starting dose

Answer 17

1.25 mg/kg/min

Question 18

CI of PCAs

Answer 18

Epoprostenil: HF
Trprostinil: Child Pugh Class C

Question 19

Warning and ADR of PCAs

Answer 19

Vasodilation reactions: hypotension, flushing → Avoid sudden, large dose reductions

Rebound PH if DC suddenly

Jaw pain, infusion-site pain

Question 20

Administration of Epoprostinal

Answer 20

Protect form light → before reconstitution and during infusion

Flolan: requires ice packs for stability

Question 21

MOA of endothelia receptor antagonists

Answer 21

Block endothelin receptors pulmonary artery smooth muscles

Question 22

BBW of ERAs

Answer 22

Teratogenic (negative pregnancy test) and monthly after

Bosentan: hepatotoxicity

REMS

Question 23

ERAs types

Answer 23

Bosentan
Ambrisentan
Macitentan

Question 24

ADR and warning of ERAs

Answer 24

Hepatotixicity, ↓ Hcrt, Hb, fluid retention

HA

Question 25

Which ERA decreases effectiveness of PO contraceptives

Answer 25

Bosentan

Question 26

MOA of PDE5I

Answer 26

Increased cGMP concentrations → pulmonary vasodilation

Question 27

PDE5I indicated for PH

Answer 27

Slidenafil (Revatio) Tadalafil (Adcirca)

Question 28

Dosing of tadalafil

Answer 28

40 mg QD

Question 29

Dosing of slidinafil

Answer 29

20 mg TID

Question 30

CI of PDE5I

Answer 30

Avoid nitrates and riociguat

Question 31

ADR of PDE5I

Answer 31

Hearing loss, vision loss, NAION, hypotension, priapism, HA

Question 32

MOA of sGCs

Answer 32

Sensitizes sGC receptor for endogenous nitric oxide → ↑ cGMP → vasodilation and anti proliferative effects of pulmonary smooth muscle

Question 33

BBW of Riociguat

Answer 33

Teratogenic → Negative pregnancy test and monthly after REMS

Question 34

CI of Riociguat

Answer 34

Pregnancy, use of PDE5-i or nitrates

Question 35

ADR and Warning of Riociguat

Answer 35

HA and hypotension

Question 36

What is pulmonary fibrosis

Answer 36

Scarred and damaged tissue → dyspnea and nonproductive cough

Question 37

Drugs that slow the rate of worsening pulmonary fibrosis

Answer 37

Pirfenidone (Esbriet) Nintedanib (Ofev)

Question 38

Drugs that can cause pulmonary fibrosis

Answer 38

Amiodarone/dronedarone Bleomycin Busulfam Carmustine