Pulmonary Arterial HTN (PAH)

Question 1

What’s the INR goal in pt with PAH using warfarin?

Answer 1

INR 1.5-2.5

Question 2

MOA of prostacyclin analogues (or Prostanoids)?

Answer 2

Potent vasodilators on pulmonary and systemic vascular beds.

They also inhibit platelet aggregation.

Question 3

List prostacyclin analogues (or Prostanoids) agents.

Answer 3

Epoprostenol (Flolan, Veletri)

Treprostinil (Remodulin - SC/IV; Tyvaso - inhaled)

Iloprost (Ventavis)

Question 4

SEs that may occur during dose titration of prostacyclin analogues (or Prostanoids)?

Answer 4

VD (hypotension, HA, flushing)
Nausea/v/d
Anxiety
Chest pains/palpitations
Tachycardia
Edema
Jaw claudication

Question 5

SEs that may occur during chronic use of prostacyclin analogues (or Prostanoids)?

Answer 5

Anxiety

Flu-like sx

Jaw pain

Thrombocytopeni

Neuropathy

Question 6

Best to avoid interruption to Prostacyclin analogues (or Prostanoids) therapy?

Answer 6

Yes

Question 7

Best to avoid large, sudden reduction to Prostacyclin analogues (or Prostanoids) dose?

Answer 7

T

Question 8

Howz Flolan pump stored?

Answer 8

Needs to be on ice packs for proper cooling

Question 9

Which agents are most potent of all PAH meds?

Answer 9

Parenteral Prostacyclin analogues (or Prostanoids) agents?

Question 10

MOA of Endothelin Receptor Anatagonists (ERAs)?

Answer 10

Blocks Endothelin (VC with cellular proliferation)

Question 11

Endothelin Receptor Anatagonists (ERAs) agents?

Answer 11

Bosentan (Tracleer) - REMS drug

Ambrisentan (Letairis) - REMS drug

Macitentan (Opsumit) - REMS drug

Question 12

BBW of Bosentan (Tracleer) - REMS drug and Ambrisentan (Letairis) - REMS drug and Macitentan (Opsumit) - REMS drug?

Answer 12

Preg cat X

Hepatoxicity (Bosentan (Tracleer) - REMS drug)

Question 13

SEs of Bosentan (Tracleer)

Answer 13

HA

High hemoglobin

High LFTs

Question 14

SEs of Ambrisentan (Letairis) - REMS drug

Answer 14

Peripheral edema

HA

Reduced hemoglobin

Question 15

SEs of Macitentan (Opsumit) - REMS drug

Answer 15

Reduced hemoglobin

HA

Question 16

List PDE-5 used in PAH?

Answer 16

Sildenafil (Revatio)

Tadalafil (Adcirca)

Question 17

CI to PDE-5 agents use?

Answer 17

Concurrent use of nitrates

Question 18

SE to PDE-5 agents use?

Answer 18

Dizziness

Sudden drop in BP

HA

Priapism

Question 19

MOA of Soluble Guanylate Cyclase (sGC) stimulator?

Answer 19

Increasing conversion of GTP to cGMP leads to increased relaxation and antiproliferative effects in the pulmonary artery smooth muscle cells

Question 20

Agents under Soluble Guanylate Cyclase (sGC) stimulator? What’s unique about it?

Answer 20

Riociguat (Adempas) - REMS drugs

Only 1 approved for both PAH and CTEPH (all others are only used for PAH)

Question 21

BBW of Riociguat (Adempas) - REMS drugs?

Answer 21

Cat x

Question 22

CI of Riociguat (Adempas) - REMS drugs?

Answer 22

Pregnancy

Concomitant use of PDE-5 inh or Nitrates

Question 23

SE of Riociguat (Adempas) - REMS drugs

Answer 23

HA

Dyspepsia

Dizziness

Hypotension

Question 24

List clinical classification of Pulmonary HTN

Answer 24

Pulmonary arterial HTN (PAH)

Pulmonary HTN owing to a left heart dx

Pulmonary HTN owing to lung dx and/or hypoxia

Chronic thromboembolic pulmonary HTN (CTEPH)

Pulmonary HTN with unclear multifactorial mechanisms