Term
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Definition
- PH - abnormally high pulmonary vascular pressure
- Pulmonary arterial hypertension - idiopathic, heritable, drug induced. Restricted blood flow through pulmonary artery leads to right heart failure |
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Term
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Definition
- Definite: Anorexigens pulled off the market, toxic rapeseed oil
- Likely - Tryptophan, amphetamines
- Possible - cocaine, chemo |
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Term
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Definition
| - Pulmonary vascular resistance (PVR) is elevated, PCWP is normal. High mean pulmonary artery pressure (mPAP) >/ 25, normal (less than 15) PCWP, high PVR (>3 wood units, 240 dyne) |
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Term
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Definition
- Multiple hit - predisposition and a stimuli
- Heritable: BMPR2 and activin-like Kinase
- Drug induced
- Constriction, inflammation, prothrombotic states |
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Term
| What levels are increased in PAH, and thus are targets of drugs? |
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Definition
- Increased Endothelin-1 (ET-1) - Vasoconstrictor
- Increased Serotonin (5-HT) - Vasoconstrictor
- Increased Thromboxane A2 - Vasoconstrictor and platelet activator
- Decreased Prostacyclin - vasodilator and prevents platelet aggregation
- Decrease nitrous oxide - vasodilator |
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Term
| What are symptoms of PAH? |
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Definition
| SOB on exertion, fatigue, SOB at rest, chest pain, syncope --> leg swelling, anorexia |
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Term
| What are the WHO functional classes of PAH? |
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Definition
Class I - no limitation of physical activity
Class II - Mild limitation, no discomfort at rest
Class III - Marked limitation, no discomfort at rest
Class IV - Unable to perform physical activity, SOB present at rest |
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Term
| What is the Clinical presentation of PAH? |
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Definition
S4 may be present, fluid overload similar to HF, cool extremities, low O2 sat
Diagnosis: Right Heart catheterization is the gold standard
ECG to see RV enlargement |
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Term
| What nonpharmacologic treatments are recommended for PAH? |
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Definition
- Low grade exercise and low salt diet
- Avoid pregnancy
- Flu and pneumonia vaccine
- Anemia management |
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Term
| What Rx treatments are recommended for all PAH patients |
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Definition
- Warfarin therapy with a goal INR of 1.5-2.5
- Diuretics to treat fluid overload
- Oxygen to increase Osat to above 90%
- No clear benefit for digoxin |
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Term
| How do you tell if a patient would respond favorably to a CCB, and what do you use for CCB therapy? |
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Definition
- Acute vasodilator testing - inhaled NO 20-40 ppm x5 min, measure hemodynamics. A positive response is a 10 or more drop in mPAP and must be less than 40.
Drug of choice is then DHP CCBs - Nifedipine XL and Amlodipine. Can use diltiazem but avoid verapamil |
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Term
| What PDE-5 inhibitors can be used and when? |
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Definition
Use when not a candidate for CCBs
Use in classes 2-4
Sildenafil/Revatio (first choice in women) and Tadalafil/Adcirca - increase cGMP
Avoid with CYP3A4 inhibitors - itraconazole and ketaconazole, NITRATES
Causes HA, flushing, loss of hearing or vision |
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Term
| What drugs are Endothelin Receptor Antagonists and when are they used? |
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Definition
Used in classes 2-4
Bosentan/Tracleer and Ambrisentan/Letairis - block endothelin receptor to lower mPAP
Both have access programs due to pregnancy category X, use 2 forms of birth control |
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Term
| What drugs are prostacyclin analogs and when are they used? |
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Definition
Used only in classes 3 and 4
Epoprostenol/Flolan or Veletri, Treprostinil/Remodulin and Tyvaso, Iloprost/Ventavis (inhaled only)
Synthetic prostacyclins, monitor ppl on anticoagulants
Epoprostanol/Flolan drug of choice in Class 4, continuous IV flow. Avoid abrupt withdrawal in all analogs |
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Term
| What are monitoring parameters for PAH progress? |
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Definition
6 minute walk distance
ECG
right-heart Cath
WHO classification |
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