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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?

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
Prostacyclin analogue dosing
Epoprostenol and treprostinil (remodulin) - ng/kg/min | Treprostinil (Tyvaso/Orenitram), Iloprost, Selexipag - mcg
26
What effect does gemfibrozil have on treprostinil levels?
increases
27
What effect does rifampin have on treprostinil levels?
decreases
28
What medications are endothelin receptor antagonists
Bosentan Ambrisentan Macitentan
29
Endothelin receptor antagonists MOA
block endothelin receptors on pulmonary artery smooth muscle cells causing vasodilation
30
Endothelin MOA
Vasoconstrictor with cellular proliferative effects
31
Endothelin receptor antagonist BBW, CI, Warnings, SE
BBW: teratogenic (neg preg test monthly); bosentan - hepatotoxicity CI: pregnancy Warnings: hepatotoxicity, decreased Hgb/Hct, fluid retention SE: HA
32
What PAH medications are only available through individual REMS programs?
Endothelin receptor antagonists (ERA) | sGC stimulator - riociguat
33
What PAH medication can decrease effectiveness of hormonal contraceptives?
Bosentan
34
What medications are PDE-5 inhibitors and are used in PAH
Sildenafil (Revatio) | Tadalafil (Adcirca)
35
PDE-5 inhibitor medications and indications
Sildenafil (Revatio) - PAH Sildenafil (Viagra) - ED Tadalafil (Adcirca) - PAH Tadalafil (Cialis) - ED
36
PDE-5 inhibitor CI, Warnings, SE
CI: use with nitrates or riociguat Warnings: hearing loss, vision loss (nonarteritic anterior ischemic optic neuropathy or NAION), hypotension, priapism SE: HA
37
PDE-5 MOA
degrades cyclic guanosine monophosphate (cGMP) preventing vasodilation
38
PDE-5 inhibitor MOA
prevents degradation of cyclic guanosine monophosphate (cGMP) causing pulmonary vasculature relaxation and vasodilation
39
Soluble guanylate cyclase stimulator (sGC) MOA
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
What medications are soluble guanylate cyclase (sGC) stimulators?
Riociguat
41
Riociguat BBW, CI, Warnings, SE
BBW: teratogenic (neg preg test monthly) CI: pregnancy, use of PDE-5 inhibitors or nitrates Warnings: hypotension SE: HA
42
What PAH medications cannot be used together
PDE-5 inhibitors and sGC stimulator Riociguat d/t hypotension
43
How does smoking cessation affect riociguat?
Smoking cessation may decrease riociguat clearance - dose may need to be decreased
44
What is pulmonary fibrosis?
scarred and damaged lung tissue
45
What drugs can cause pulmonary fibrosis?
``` Amiodarone Dronedarone Bleomycin Busulfan Carmustine Lomustine Nitrofurantoin Sulfasalazine ```
46
Treatment options for pulmonary fibrosis
D/c offending agent/medication Oxygen supplementation Pirfenidone (Esbriet) and Nintedanib (Ofev) can slow progression of pulmonary fibrosis

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