Pulmonary Arterial Hypertension Flashcards
What is Pulmonary Arterial Hypertension [PAH]?
- Continuous high blood pressure within the pulmonary arteries [mPAP > 25]
What are some drugs that can cause PAH?
- Cocaine
- Fenflramine
- Meth
- SSRIs during pregnancy
- Weight loss drugs
What is the Pathophysiology of PAH?
- Imbalence of vasocontrictors [endothelin-1, Thromboxane A2 = increased] and vasodilators [Prostacyclin = decreased]
- This causes imbalences between cell growth and death = scarring of the artery smooth muscle = constriction
What is the most common cause of death for those that have PAH?
- Heart Failure
What are some of the symptoms of PAH?
- Fatigue, Dyspnea, Chest Pain, Fainting, Edema, Tachycardia, Raynauds
What are some of the non-drug treatments for PAH?
- Sodium restricted diet of < 2.4 g/day
- Avoid drugs that increase sodium/H20 retention [NSAIDS]
- Get Flu and Pneumona vaccines
What is the way that doctors confirmed the diagnosis of PAH?
- Right heat cath with short acting vasodilators [responder or non-responder]
What does it mean when a patient is a responder to the Right Heart Cath in PAH?
- mPAP is falling by 10 to less than 40 mmHg
- Treat with CCB [Nifedipine, Diltiazem, Amlodpine]
NO Verapamil as it has more (-) inotropic effects
What does it mean when the patient is a non-responder to the Right Heart Cath in PAH?
- (+) responders and those that fail CCB
- Treat with Prostacycline Analogs, ERAs, PDE-5i, sGC
What are some important supportive therapies that can be used in PAH?
- Loop Diuretics [volume overload]
- Digoxin [cardiac output]
- Warfarin [anti-coag]
What is the way that the Prostacyclin Analogues [or prostanoids] and Receptor Agonist work to help with PAH?
- PAH reduces Prostacyclin = decrease Prostacyclin I2 [vasodilator]
- Prostanoids are potent vasodilators & inhibit platelet aggregation
IV INFUSION AT HOME
What are the Prostanoids that are used for PAH?
- Epoprostenol [Flolan] - Continuous IV Infusion
What are the Side Effects for Eproprostenol in PAH?
- HYPOtension
- Flushing
- Jaw Pain
- Infusion-site Pain
What are the Warnings for Eproprostenol in PAH?
- Vasodilation Reactions [HYPOtension, Flushing]
- Rebound PH [when large changes in drug]
- Since IV Infusion = increase infection risk
What are some additional notes for Eproprostenol in PAH?
- Flolan is a very potent vasodilator [avoid large/sudden dose changes]
- Flolan MUST be protected from light and reconstitued with an ince pack
Example Calculation Question
AM is a 42 year old female [weight 85kg] in the ICU with PAH. At home she mixes 9.5 ml of 1mg/mL treporstinil [Remodulin] with 40.5 mL of sterile water. She sets her pump to 0.06mL/hr. what is her dose in ng/ki/min?
do if want too??
- 2.2 ng/kg/min
What is the way that Endothelin Receptor Antagonists [ERAs] work to help with PAH?
- ERAs block endothelin [a vasoconstrictor] to help increase vasodilation
What are the ERAs that are used for PAH?
- Bosentan [Tracleer]
- Ambrisentan [Letaniris]
- Macitentan [Opsumit]
What are the BOXED WARNINGS for ERAs in PAH?
- Teratogenic [REMS] - needs (-) pregnancy test before start and monthly after
- Hepatotoxicity
What are the Contraindications for ERAs in PAH?
- Pregnancy
What are the Warnings for ERAs in PAH?
- Hepatotoxicity
- Decreased Hgb/Hct
- Fluid Retention [edema like]
What are the Side Effects for ERAs in PAH?
- Headache, Upper RTI, Flushing, Hyportension
What is the way that the Phosphodiesterase-5 Inhibitors work within the body for PAH?
- PDE-5 degradates cGMP; inhibiting PDE-5 will increase cGMP = vasculature relaxation and diliation
What are the PDE-5 Inhibitors that are used in PAH?
- Sidenafil [Revatio] (Viagra = ED)
- Tadalafil [Adcirca] (Cialis = ED & BPH)
What are the Contraindications for PDE-5 Inhibitor in PAH?
- AVOID with Nirtates or Riociguat
What are the Warnings for PDE-5 Inhibitor in PAH?
- Hearing Loss [w/ or w/o Tinnitus]
- Vision Loss [Nonarteritic Anterior Ischemic Optic Neuropathy (NAION)]
- HYPOtension
- Priapism
What are the Side Effects for PDE-5 Inhibitor in PAH?
- Headache
- Flushing
- Back Pain
- N/D
What is the way that the Soluble Guanylate Cyclas Stimulator [sGC stims] work within PAH?
- sGC is a receptor for Nirtic Oxide; sGC stims sensitizes cGMP and stimulates the receptor
- This increases cGMP = Relaxation
What are the sGC Stims that are used in PAH?
- Riociguat [Adempas]
What are the BOXED WARNINGS for Riociguat in PAH?
- Teratogenic [REMS] - needs (-) pregnancy test before start and monthly after
What are the Contraindictaions for Riociguat in PAH?
- Pregnancy
- Use of PDE-5i or nitrates
What are the Warnings for Riociguat in PAH?
- HYPOtension
- Bleeding
- Pulmonary Edema
What are the Side Effects for Riociguat in PAH?
- Headache
- Dyspepsia [indegestion]
- Dizziness
- N/V/D
What is Pulmoary Fibrosis?
- Scarring and Damage of Lung Tissue
What are some drugs that can cause Pulmonary Fibrosis?
- Amiodarone
- Bleomycin
- Busulfan
- Carmustine
