Term
| Prostacyclin (PGI-2) on smooth muscle cells |
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Definition
| causes muscle relaxation, vasodilation, inhibits muscle proliferation |
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Term
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Definition
| inhibits platelet aggregation |
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Term
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Definition
| produced by activated platelets as well as endothelial cells |
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Term
| Thromboxane A2 on smooth muscle cells |
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Definition
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Term
| Thromboxane A2 on platelets |
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Definition
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Term
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Definition
| Epoprostenol, Treprostinil, Iloprost |
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Term
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Definition
only drug shown to improve mortality
short t1/2, lack of stability, given as continuous infusion, expensive
SE: HA, Flushing, jaw pain, diarrhea |
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Term
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Definition
longer t1/2, stable at rt, given IV or subQ,
4x/day, indicated for less severe PAH |
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Term
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Definition
| Only approved as monotherapy in treatment-naive patients with PAH |
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Term
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Definition
synthetic analog, inhalation, short t1/2
6-9x/day, causes CG & bronchoconstriction becuase it sensitizes airway sensory nerves |
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Term
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Definition
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Term
|
Definition
longer t1/2 7.9 hrs
SE: HA,Diarrhea, Nausea |
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Term
Endothelin receptor A
(ET-A) |
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Definition
| mainly expressed in Pulmonary vascular smooth muscles, contribute to ET-1 induced contraction and proleferation |
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Term
Endothelin receptor B
(ET-B) |
|
Definition
expressed in endothelial cells
lead to ET-1 clearance and vasodilation |
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Term
|
Definition
| both expressed on smooth muscle cells but ET-A dominates |
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Term
|
Definition
| Bosentan, Ambrisentan, Macitentan |
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Term
|
Definition
Non selective antagonist, improves mortality, abnormal liver function test, potential teratogenic,
improve 6 min walk test |
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Term
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Definition
| selective for ET-A, low risk for liver toxicity and drug-drug interaction, peripheral edema. teratogenic |
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Term
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Definition
| non selective antagonist, anemia, low liver toxicity, teratogenic and contraindicated in pregnancy |
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Term
Nitric oxide Agents
PDE5-I |
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Definition
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Term
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Definition
elevate cGAMP, vasodilatory, antiproliferative effects
IV injection and oral (20mg 3x/day lower than erectile dysfunction dose) |
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Term
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Definition
t1/2-17.5 hrs, 1x/day,elevate cGAMP, vasodilatory, antiproliferative effects
improve 6min walk test |
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Term
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Definition
stimulates soluble Guanylate cyclase
oral 3x/day, combining with PDE5-I is potentially harmful |
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