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Epoprostenol (Flolan®) Prostacyclin Analogues for class III and first line class IV and catheter SE: Nausea, vomiting, headache, jaw pain, flushing, hypotension, bradycardia, reflex tachycardia (at higher doses), flu-like symptoms Antiplatelet effect Line infections, sepsis, catheter obstruction CI: Development of pulmonary edema, CHF due to severe L ventricular systolic dysfunction Portable infusion pump --- pump failure ordislodging can result in rebound pulmonary hypertension and possible death medical emergency unstable at room temperature a Treprostinil SQ or IV no oral class 2-4 SE: Nausea, vomiting, headache, jaw pain, flushing, hypotension, bradycardia, reflex tachycardia (at higher doses), flu-like symptoms Antiplatelet effect Line infections, sepsis, catheter obstruction (with IV formulation), infusion-site pain/swelling (with SQ formulation) Safer, more convenient, and less temperature-labile than epoprostenol Portable pump inhaled treprostini class 3 SE same as others, pro throat irritation cough lloprost protable neubulizer /inhale class 3 and 4 syncope SBP<85mmHg cough,flusing,flu-like syndrome,jaw pain, bronchospasms, antiplatlet effects neb adaptive aerosol delivery system sildenafil/tadalafil act like NO sustain cGMP class 1,2,3 cause flushing, HA, heartburn, muscle aches, vision (rare). vasorelaxation Bosentan 2C9 3A4 endotherial receptotr antagonist 2-4 FDA black box warning for hepatic toxicity, monthly LFTs Pregnancy category X monthly pregnancy test, two methods of contraception (except IUD) during and one month after stopping bosentan therapy in females of childbearing potential Headache, flushing, peripheral edema, decreased sperm count, angioedema Anemia: monitor hematocrit every 3 months ambrisentan 3A4 2C19 endptherial receptor antgaonist adjunct therapies treatment of HF digoxin diuretics oxygen chronic anticoagulation warfarin limit Na intake and get immunized