PAH

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Author:
DrJBlack
ID:
137116
Filename:
PAH
Updated:
2012-02-23 00:40:55
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Pulmonary Arterial HTN
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Description:
PAH for Cardio Exam #2
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  1. PAH Charcteristics
    • rare but progressive lung disease that leads to right-sided heart failure and premature
    • no cure
    • Definition: it's sustained elevated pulmonary arterial pressure of > 25 mmHg at rest; > 30 mmHG during exercise; mean pulmonary capillary wedge pressure and left ventricular end-diastolic pressure of < 15 mmHg
  2. Pathology of PAH
    extensive vascular narrowing of pulmonary arteries due to vasconstriction, remodeling, and thrombosis --> widespread obstruction and obliteration of smallest pulmonary arteries --> sufficient number of arteries are occluded, increased resistance to pump blood into lungs --> RV attempts compensate for increased resistance by increased pressure --> hypertropy --> right-sided heart failure
  3. Main contributor to arterial damage is endothelial dysfunction which leads to
    • overproduction of vasoconstrictors: ET-1 (endothelin) and TXA2
    • impaired production of vasodialators: NO (nitric oxide) and PGI2 (prostacyclin)
    • PGI2 and TXA2 are both AA derivatives
  4. Endothlial cells
    single layer of cells lining the blood vessels that serve as one route of communication between blood and vascular smooth muscle
  5. Other roles of endothelial cells
    • NO
    • synthesize humural mediators
    • antithrombotic activity (fight platelet aggregation)
    • regulates mitosis and development of vascular smooth muscle
  6. PGI2
    • prostacyclin
    • potent vasodialator of both pulmonary and systemic ciruclations, and has antiplatelet activity
  7. NO
    • nitric oxide
    • potent vasodialators and inhibits platelet adhesions
  8. TXA2
    potent vasoconstriction and promotes platelet aggregation
  9. ET-1
    • endothelin 1
    • potent vasoconstrictor via endothelin receptor A (ETa)
    • smooth-muscle mitogen (ETa)
    • promotes release of PGI2 & NO from pulmonary endothelial cells (ETb)
  10. Causes of PAH
    • idiopathic (40%)
    • disease-induced: pulmonary/hepatic
    • drug induced: cocaine/amphetamine; Fen Phen
  11. PAH patient characteristics
    • more common in women with typical age of diagnosis is 20-40 years (mean survival age 2.4 years)
    • likely to present with a hx of snoring and sleep-disoredered breathing
    • overproduction of vasoconstrictors and impaired production of vasodialators --> vasoconstriction > vasodialation
  12. Presenting Symptoms of PAH (FE-WAS)
    • Fatigue
    • Exertional dyspnea
    • Weakness
    • Anginal Pain
    • Syncope
  13. Disease Progression of PAH (LAA)
    • leg swelling
    • abdominal bloating & distension
    • anorexia
  14. Advanced PAH disease
    • diastolic murmur of pulmonary regurgitation
    • holosystolic murmur of tricuspid regurgitation
    • hepatojugular reflux
    • pulsatile liver
    • jugular vein distension
    • peripheral edema
    • rales and wheezing
  15. Class I WHO
    • no limitation of physical activity; then from here, you progressively get worse
    • based upon limits of physical activity
  16. 6MWT
    6-minute walk test measures clinical response to therapy and is used to determine functional decline over time and remains the only FDA-accepted exercise end point evaluating the effects of studied therapies in PAH
  17. Prostacyclin Analogs
    • Epoprostenol, Iloprost, Treprostinil
    • MOA: direct dialation of pulmonary and systemic arterioles & inhibits platelet aggregation
    • SE: flushing, HA, nausea, muscle bone/pain generally in the jaw or foot (Iloprost has jaw spasms)
    • DI: diuretics, anti-coagulants, vasodialators, anti-hypertensives, anti-platelet, digoxin (DAVAAD) --> concerned about hypotension and bleeding
  18. Eproprostenol
    • Half-life ~6 min
    • Route: continuous IV infusion via implanted delivery system thru indwelling central venous catheter (permanent)
    • Additional SE: hypotension and sepsis
    • Misc: once reconsitituted, not stable at RT (must refrigerate); must store in fridge and use ice packs around pump; also requires anti-coagulants unless CI
    • Advantages: 1st line III and IV
    • Disadvantages: route, handling, sepsis
  19. Treprostinil (SQ)
    • Half-life: 45 min IV or 3 hr SQ; half-life depends on route
    • Route: continuous infusion thru at abdominal SQ pump
    • Additional SE: pain & erythema at SQ infusion site
    • Misc: stable at room temperature (lower infection risk); commercially available as a premixed syringe --> increase sterility
    • Advantages: 2nd line therapy III; better than IV
    • Disadvantages: pain and erythema at SQ infection site; route
  20. Treprostinil (Inh)
    • Route: inhalation via nebulizer device (9 breaths/session, QID) titrate up from 3 breaths per session)
    • Addtional SE: cough, throat irritation, syncope
    • Misc: the daily doses should be equally spaced and during waking hours only q4h
    • Advantages: route
    • Disadvantage: dosing; algorithim rank
  21. Treprostinil IV
    • Advantages: lower infection risk. 3rd line III, stable at RT, premix syringe --> sterility
    • Disadvantages: route; 4th line IV
  22. Iloprost
    • half-life - 20-30 min
    • Route: inhalation via nebulizer (6-9 inhalations/day during waking hours)
    • Additional SE: transient cough, hypotension, jaw muscles spasms
    • Misc: need at least 2 hours b/t doses; effects resolve 20-90 min after inhalation
    • Advantages: 1st line III; 2nd line IV; route; success
    • Disadvantages: jaw muscle spasms; 6-9 doses have to wait at least 2 hrs
  23. Agent preference of prostacyclin analogs
    IV epoprostenol or inahled iloprost > SQ treprostinil > IV Treprostinil or IV Iloprost > inhaled treprostinil
  24. Endothelin receptor antagonists
    • Bosentan and Ambrisentan
    • SE: HA, flushing, leg edema, anemia --> decreased Hb and Hct
    • BBW: severe hepatoxicity for bosentan (NOT ambrisentan); teratogenicity - precuations include negative pregnancy tests before drug therapy
    • while on drug therapy you must take 2 forms of contraceptives (barrier and hormonal) and monthly pregnancy tests
    • Exception: tubal ligation or coppoer or progestin IUD
    • Follow up: continue contraceptives for one month after d/c
    • Drug Interactions: both agenst are metabolized by CYP3A4 and 2C9; both enhance metabolism of hormonal contraceptives, but ambrisentan to lesser extent
  25. Bosentan
    • MOA: competive antagonist at ET-1 receptor types ETa and ETb
    • Adminstration: po BID (independent of meals)
    • Special Considerations: tracleer access program (TAP) restricted distribution, use and prescribing plan due to potential toxicities
    • DI: warfarin and sildenafil
    • Advantages: oral BID; 1st line II & III; w/o meals
    • Disadvantages: DI; severe hepatoxicity; restricted access; 2 forms of contraceptives; monthly pregnancy tests; teratogencity
  26. Ambrisentan
    • MOA: specific and competitive antagonist at ETa recpetors
    • Administered PO QD (independent of meals)
    • Special considerations: Letaris Education and Access Program (LEAP)
    • Advantages: QD independent of meals; 1st line II and III; oral route; lesser SE, DI, no hepatoxicity
    • Disadvantages: 2 forms of contraceptives; teratogenicity; monthly pregnancies; resticted access
  27. Type 5 PDE Inhibitors
    • sildenafil > tadalafil
    • MOA: increase or prolong NO and increase cGMP in vascular smooth muscle
  28. Sildenafil
    • Advantages: 1st line II and III; oral route; more preferred
    • Disadvantages: TID
  29. Tadalafil
    • Advantages: 2nd line II and III; QD; oral
    • Disadvantage: less preferred; not first line; negative connotation
  30. Traditional/Supportive therapy
    • tx of sleep apnea & obstructive sleep related disorders
    • tx of anxiety and depression
    • recommend immunizations and avoidance of pregnancy
    • oxygen
    • warfarin
    • diruretics
    • CCBs - 1st line therapy
    • digoxin
  31. Lung transplant
    • mortality rate of 16-29% and five year survival of ~45%
    • last resort
  32. Class II
    • supportive care + ET-1 antagonist or PDEI
    • all oral
    • sildenafil > tadalafil
  33. Class III
    • supportive care + ET-1 antagoinist/PGI2 analog/PDE1
    • combo of 2 when deteriorate or no improvement
  34. Class IV
    • supportive care + PGI2
    • add PGI2 or ET-1 antagonist (can go thru all 3 PGI2 or can try ET-1 antagonist)
    • Add PGI2 and ET-1 antagonist
    • PDEI = last resort

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