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NAPLEX Review > Pulmonary Arterial Hypertension > Flashcards

Pulmonary Arterial Hypertension Flashcards

1
Q

Normal pulmonary arterial pressure (mPAP or PAP)

A

8-20 mmHg when resting

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2
Q

What pulmonary arterial pressure (mPAP or PAP) is considered pulmonary hypertension?

A

25 mmHg or greater

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3
Q

What are the 5 groups of pulmonary arterial hypertension (PAH)?

A

1: idiopathic, heritable, drug and toxin-induced, disease associated and PAH in newborn
2: left heart disease
3: lung disease and/or hypoxia
4: chronic thromboembolic pulmonary hypertension (CTEPH)
5: unclear or multifactorial mechanism

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4
Q

How to treat each group of PAH?

A

Group 1: with medications discussed here (idiopathic PAH)

Group 2-5: treat underlying cause

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5
Q

What medications can cause PAH?

A 
Cocaine
SSRI during pregnancy increased risk for newborn
Weight loss drugs
Methamphetamines/Amphetamines
Dasatinib
Diazoxide
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6
Q

Does endothelin-1 cause vasodilation of vasoconstriction?

A

Vasoconstriction

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7
Q

Does thromboxane A2 (TXA2) cause vasodilation of vasoconstriction?

A

Vasoconstriction

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8
Q

Do prostacyclins cause vasodilation of vasoconstriction?

A

Vasodilation

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9
Q

What is the most common cause of death in patients with PAH?

A

Heart failure

Caused because the heart has to work harder and the right ventricle becomes enlarged

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10
Q

What is the life expectancy for someone with untreated PAH?

A

3 years

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11
Q

Non-pharm treatment options for PAH

A 
Sodium restricted to <2.4g/d
Avoid NSAIDs
Flu and pneumococcal faccines recommended
Avoid high altitudes
Oxygen when O2 sats fall below 90%
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12
Q

How is PAH diagnosed?

A

Right heart catheterization

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13
Q

How should you treat someone with PAH that is determined to be a responder?

A

Oral CCB

If response is not sustained, begin PDE-5i, ERA, sGS stimulator, prostacyclin analogue

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14
Q

How should you treat someone with PAH that is determined to be a non-responder?

A

PDE-5i
Endothelin receptor antagonist (ERA)
Soluble guanylate cyclase (sGC) stimulator
Prostacyclin analogue

If inadequate response - add additional meds or titrate current meds

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15
Q

What CCBs are used for PAH

A

Long-acting

Nifedipine, diltiazem, amlodipine

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16
Q

What CCB should NOT be used for PAH?

A

Verapamil - has negative inotropic effects

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17
Q

What prostacyclin analogue reduces mortality?

A

Epoprostenol

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18
Q

What are supportive therapies for PAH?

A

Loop diuretics (volume overload)
Digoxin (improves CO or rate control)
Warfarin (pts typically in pro-thrombotic state)
Oxygen

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19
Q

What INR goal should patients with PAH have?

A

1.5-2.5

20
Q

What natural product is reduced in PAH?

A

Prostacyclin synthase - decreased production of prostacyclin I2

21
Q

Prostacyclin analogues (prostanoids) MOA

A

inhibit platelet aggregation and cause potent vasodilation

22
Q

What medications are prostacyclin analogues?

A

Epoprostenol (Flolan) (continuous IV infusion)
Treprostinil
Iloprost
Selexipag

23
Q

Prostacyclin analogue warnings and SE

A

Warnings: Vasodiation reaction, rebound PH (can be fatal), blood stream infections with chronic IV infusions
SE: Hypotension, flushing, jaw pain, infusion-site pain (for IV/SQ infusions)
Flolan, Veletri, Remodulin are parenteral and VERY potent vasodilators - avoid interruption

24
Q

Which prostacyclin analogue needs to be protected from light

A

Epoprostenol

Protect from light before reconstitution and during infusion

25
Q

Prostacyclin analogue dosing

A

Epoprostenol and treprostinil (remodulin) - ng/kg/min

Treprostinil (Tyvaso/Orenitram), Iloprost, Selexipag - mcg

26
Q

What effect does gemfibrozil have on treprostinil levels?

A

increases

27
Q

What effect does rifampin have on treprostinil levels?

A

decreases

28
Q

What medications are endothelin receptor antagonists

A

Bosentan
Ambrisentan
Macitentan

29
Q

Endothelin receptor antagonists MOA

A

block endothelin receptors on pulmonary artery smooth muscle cells causing vasodilation

30
Q

Endothelin MOA

A

Vasoconstrictor with cellular proliferative effects

31
Q

Endothelin receptor antagonist BBW, CI, Warnings, SE

A

BBW: teratogenic (neg preg test monthly); bosentan - hepatotoxicity
CI: pregnancy
Warnings: hepatotoxicity, decreased Hgb/Hct, fluid retention
SE: HA

32
Q

What PAH medications are only available through individual REMS programs?

A

Endothelin receptor antagonists (ERA)

sGC stimulator - riociguat

33
Q

What PAH medication can decrease effectiveness of hormonal contraceptives?

A

Bosentan

34
Q

What medications are PDE-5 inhibitors and are used in PAH

A

Sildenafil (Revatio)

Tadalafil (Adcirca)

35
Q

PDE-5 inhibitor medications and indications

A

Sildenafil (Revatio) - PAH
Sildenafil (Viagra) - ED
Tadalafil (Adcirca) - PAH
Tadalafil (Cialis) - ED

36
Q

PDE-5 inhibitor CI, Warnings, SE

A

CI: use with nitrates or riociguat
Warnings: hearing loss, vision loss (nonarteritic anterior ischemic optic neuropathy or NAION), hypotension, priapism
SE: HA

37
Q

PDE-5 MOA

A

degrades cyclic guanosine monophosphate (cGMP) preventing vasodilation

38
Q

PDE-5 inhibitor MOA

A

prevents degradation of cyclic guanosine monophosphate (cGMP) causing pulmonary vasculature relaxation and vasodilation

39
Q

Soluble guanylate cyclase stimulator (sGC) MOA

A

sensitizes sGC to endogenous nitric oxide and directly stimulates the receptor at a different binding site, increasing cGMP causing relaxation in pulmonary artery smooth muscle cells

40
Q

What medications are soluble guanylate cyclase (sGC) stimulators?

A

Riociguat

41
Q

Riociguat BBW, CI, Warnings, SE

A

BBW: teratogenic (neg preg test monthly)
CI: pregnancy, use of PDE-5 inhibitors or nitrates
Warnings: hypotension
SE: HA

42
Q

What PAH medications cannot be used together

A

PDE-5 inhibitors and sGC stimulator Riociguat d/t hypotension

43
Q

How does smoking cessation affect riociguat?

A

Smoking cessation may decrease riociguat clearance - dose may need to be decreased

44
Q

What is pulmonary fibrosis?

A

scarred and damaged lung tissue

45
Q

What drugs can cause pulmonary fibrosis?

A 
Amiodarone
Dronedarone
Bleomycin
Busulfan
Carmustine
Lomustine
Nitrofurantoin
Sulfasalazine
46
Q

Treatment options for pulmonary fibrosis

A

D/c offending agent/medication
Oxygen supplementation
Pirfenidone (Esbriet) and Nintedanib (Ofev) can slow progression of pulmonary fibrosis

NAPLEX Review (79 decks)

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