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19 Cards in this Set
- Front
- Back
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PAH |
High bp in pulmonaries (>25mPAP) 8-20 mmHg is normal/resting 5 groups of PAH: 1- idiopathic, drug induced, infection 2- left heart disease 3- lung disease/hypoxia 4- chronic thromboembolic(CTEPH) 5- unknown S/sx- fatigue, edema, chest pain, Raynauds phenomenon(discoloration, coldness) Pathophys- TxA and endothelin-1 increased(vasoconstrictors), prostacyclin decreased(vasodilators) |
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Drugs that cause PAH |
Cocaine Dasatinib(Sprycel) Diazoxide(Proglycem)- hyperinsulemic hypoglycemia antidote Methamphetamine SSRI during preg increase risk in newborn Weight loss agent- diethylpoprion, lorcaserin, phendimetrazine, phentermine |
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Non-drug Tx |
Low sodium diet, routine immunizations(flu and Pna) Oxygen Avoid NSAIDs |
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Tx algorithm |
1. Warfarin(INR 1.5-2.5) + loop diuretic+ oxygen + digoxin 2. Right heart catheterization-> acute vasoreactive testing(short acting vasopdilators given- inh NO, IV epoprostenol, IV adenosine); >10mmHg drop=positive 3+. Oral CCB(ER nifedipine, diltiazem, amlodipine 3-. Prostacyclin analog(IV epoprostenol) 4-. Sequential combo therapy- endothelin receptor antagonist+ prostanoids + PDE-5 inhibitor or sGC 5-. Lung transplant |
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Prostacyclin analogues/Prostanoids |
Vasodilators + lower platelet aggregation CI- liver impairment(class C)- only po treprostinil Warning(po treprostinil)- alcohol, ghost shells can get stuck in diverticuli Sfx- (during titration) NVD, vasodilation, tachycardia, edema, jaw claudication;(chronic) anxiety, flu-like s/sx, jaw pain, thrombocytopenia, neuropathy Avoid tx interruptions, large change in dose Parenteral agent most potent |
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Epoprostenol |
Flolan, Veletri IV infusion(continuous) 2ng/kg/min, increase by 1ng/kg/min in 15 min. Increments Normal is 25-40ng/kg/min to 200ng/kg/min(over years) Flolan must be cooled with ice pack and protected from light |
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Treprostinil |
Remodulin(SC/IV), Tyvaso(inh), orenitram(po) Tyvaso- causes cough Orenitram- GI effects worse than others, take with food Remodulin- very painful |
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Iloprost |
Ventavis Inh 2.5-5mcg/inh x6-9 a day |
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Endothelin receptor antagonist(ERAs) |
Bosentan(Tracleer), ambrisentan(Letairis), macitentan(Opsumit) All requires own REMS program( TAP, LEAP, Opsumit) Pregnancy caregory X All cause headache, decrease Hgb Requires neg preg test All po CYP 3A4 substrates, avoid st. John wort |
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Bosentan |
Tracleer 62.5mg po BID; >40kg, increase to 125mg BID after 4 wks Boxed warning- hepatotoxic CI- cyclosporine, glyburide Sfx- higher LFT, URTI, edema, male infertility Monitor q mo for LFT, bilirubin; hgb and hct at 1 mo, 3mo, the q3mo |
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Ambrisentan |
Letairis 5-10mg daily CI- idiopathic pulmonary fibrosis Sfx- peripheral edema, flush, palpitation Monitor hgb and hct |
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Macitentan |
Opsumit 10mg daily Sfx- pharyngitis, anemia Monitor hgb, hct and LFT |
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PDE-5 inhibitor |
Increase cGMP- relax and vasodilation CI- nitrates, riociguat Warning- color discrimination impaired, hearing/vision loss, priapism Sfx- dizziness, HA, hypotension, flushing, back pain, epistaxis Avoid in liver impaired |
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Sildenafil |
Revatio(Viagra) 2.5-5mg TID IV 5-20mg TID po(4-6hr apart) |
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Tadalafil |
Adcirca(cialis) 40mg daily; 20mg if renal or hepatic impairment CrCl <30 avoid |
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Soluble guanylate Cyclase Stimulator |
Riociguat(Adempas) Also for CTEPH Increases cGMP via GTP 2.5mg po TID target Boxed- pregnancy X, REMS program CI- pregnancy, PDE-5inhibitors, nitrates Sfx- HA, dyspepsia, dizziness, NVD, hypotension Separate antacid by 1hr Higher dose if smoking actively |
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Pulmonary fibrosis |
Also called usual interstitial pneumonia S/sx- dyspnea w/ no productuve cough Cause- toxin, drugs( amiodarone, methotrexate, nitrofurantion, sulfasalazine), med condition Drugs for PAH can be used |
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Pirfenidone |
Esbriet Take with food, po Sfx- NVD, rash, photosensitivity Monitor LFT q mo for 6 mo, then q3 mo |
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Nintedanib |
Ofev 150mg q12h po Take with food and whole glass of water Warning- increase bleeding Sfx- NVD, increase LFT Monitor LFT Preg D Hazardous drug- needs proper handling |
