Pulmonary Arterial Hypertension Flashcards

1
Q

What agents are used to treat PAH?

A

dihydropyridines
sildenafil
prostacyclin analogs
endothelin antagonists

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2
Q

What are the 3 forms of PAH?

A

idiopathic
familial
associated

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3
Q

What is the most common form of PAH?

A

associated

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4
Q

What are the potential causes of associated PAH?

A
connective tissue disease
congenital heart disease
portal hypertension
HIV
schistosomiasis
sickle cell disease
anorexic drugs
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5
Q

What are the clinical presentations of PAH?

A

exertional dyspnea, fatigue
lower leg edema, bloating, anorexia
heart murmmers, valve disease

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6
Q

How do you confirm diagnosis of PAH?

A

right heart catheterization

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7
Q

What are the goals of PAH treatment?

A

alleviate symptoms/ improve QoL
prevent disease progression
improve survival

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8
Q

What are some nonpharmacological treatment options for PAH?

A

CPAP for sleep apnea
surgery
reduce Na intake

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9
Q

What are the general pharmacological treatments for PAH?

A

anticoagulants
diuretics
oxygen
digoxin

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10
Q

Which CCBs are indicated for PAH?

A

dihydropyridines

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11
Q

What class does sildenafil fall under?

A

phosphodiesterase inhibitors

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12
Q

Why are phosphodiesterase inhibitors used?

A

increase cGMP mediated vasodilation

improve exercise tolerance

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13
Q

What effects does prostacyclin have?

A

vasodilator
platelet inhibitor
antiproliferative

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14
Q

What is epoprostenol (Flolan)?

A

synthetic analog

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15
Q

What is treprostinil (Remodulin)?

A

stable analog

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16
Q

What is Endothelin?

A

potent vasoconstrictor and VSM mitogen

17
Q

How do endothelin antagonists treat PAH?

A

improve exercise capacity
hemodynamics
slow disease progression

18
Q

What are the endothelin antagonist agents?

A

bosentan (Tracleer)
ambrisentan (Letairis)
macitentan (Opsumit)

19
Q

What pregnancy category are the endothelin antagonists?

A

X