Pulmonary artery hypertension Flashcards

1
Q

What is PAH

A

restricted blood flow through pulmonary circulation, increased pulmonary pressure, PVR, low flow, high resistance

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2
Q

Normal Mean pulmonary arterial pressure

A

14+/- 3mmHg

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3
Q

PAH classified by

A

mPAP>25mmHg at rest and >30 w/ exercise, PCWP

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4
Q

Group 1 PAH

A

idiopathic, familial, associated with drugs, toxins, congenital heart disease, portal htn, HIV, shistosomiasis, connective tissue disease

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5
Q

Group 2 PAH

A

left sided hf, valvular dysfunction

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6
Q

Group 3 PAH

A

respiratory disease, COPD or interstitial lung disease

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7
Q

Group 4 PAH

A

thromboembolic disease

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8
Q

Group 5 PAH

A

Miscellaneous

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9
Q

Approved drugs are aimed to target

A

Group 1

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10
Q

Drugs that can cause PAH

A

cocaine, amphetamines, fen-fen

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11
Q

3 mechanisms of vascular injury to pulmonary arterioles

A

vasoconstriction, platelet dysfunction leading to thrombosis, vascular smooth muscle hypertrophy proliferation and hyperplasia

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12
Q

Endothlial dysfunction results from and imbalance mediators

A

dec nitric oxide synthase, dec prostacyclin, and inc endothelin 1 all lead to vasoconstriction

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13
Q

Non specific clinical features

A

dyspnea, fatigue, exertional syncope/angina, cx pain, palpitations, peripheral edema

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14
Q

Severe signs of PAH, usually when diagnosed

A

hepatomegaly, peripheral edema, weight gain from fluid, ascites, JVD

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15
Q

Ways to determine severity

A

effort tolerance, 6MWT (

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16
Q

Patients who respond to vasodilator test

A

dec of 10 mmHg from baseline and inc or unchanged CI are considered responders and are eligible for treatment with CCBs

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17
Q

Class 1 PAH

A

no resulting limitation or physical activity

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18
Q

Class 2 PAH

A

slight limitation of physical activity, comfortable at rest

19
Q

Class 3 PAH

A

marked limitation of physical activity, comfortable at rest, little activity cause symptoms

20
Q

Class 4 PAH

A

inability to carry out any physical activity w/out sx, right side HF, fatigue at rest

21
Q

Supportive care

A

diuretics, anticoagulation (1.5-2.5 INR), supplemental O2, digoxin

22
Q

CCBs

A

only systemic anti-HTN to show benefit, high dose in absence of HF, Diltiazem if pt tachy, nifedipine or amlodipine if brady

23
Q

PAH specific therapies

A

Prostanoids, endothelin recepto-1 antagonists, PDE-5 inhibitors

24
Q

Prostanoids

A

Epoprostenol (Flolan, Veletri), Treprostinil (Tyvaso, Remodulin), Iloprost (Ventavis)

25
Q

Endothelin Receptor-1 antagonist

A

Bosentan (Tracleer), Ambrisentan (Letairis)

26
Q

PDE-5 inhibitors

A

Sildinafil (Ravatio), Tadalafil (Adcirca)

27
Q

MOA of prostanoids

A

strong vasodilator of all vascular beds and potent endogenous inhibitor of platelet aggregation

28
Q

Adverse effects of Epoprostenol (Flolan, Veletri)

A

flushing, HA, hypotension, pump malfunction, catheter related infections, thrombosis

29
Q

Treprostinil (Remodulin)

A

develop in response to Epoprostenol, continuous SC* or IV, T1/2 is 4 hours and does not require protection from light or heat, no reconstitution

30
Q

Epoprostenol (Flolan, Veletri) negatives

A

very short T1/2 (3-5 min), must be constituted, must be kept cool

31
Q

Treprostinil (Remodulin) ADRs

A

Pain at injection sight, flushing, HA, hypotension

32
Q

Iloprost (Ventavis)

A

administered inhalation by I-neb or Prodose, 6-9 times per day, treatment takes 10 mins, ADRs are minimal

33
Q

Difference between I-neb and Prodose

A

I-neb is hand-held, battery operated, 2 methods of inhalation, $$$, requires daily cleaning, Prodose is cheaper and less portable

34
Q

Treprostinil (Tyvaso) inhaler

A

approved in 2009, effective as Iloprost, longer half life, faster administration, similar ADRs

35
Q

Bosentan (Tracleer)

A

non selective competitive antagonist for ETa and ETb, functional class 2-4, oral, inc AST/ALT, anemia, risk of tratogenicity, restricted access (TAP)

36
Q

Ambrisentan (Letairis)

A

Selective ETa receptor agonist, functional class 2-3, oral, less liver toxicity, risk of teratogenicity, anemia, restricted access (LEAP)

37
Q

MOA of endothelin-1 receptor antagonist

A

inhibit vasoconstriction and smooth muscle proliferation caused by endothelin, weak efficacy compared to Prostanoids

38
Q

Negatives of ERAs

A

monitor LFTs, HCG, both contraindicated in pregnancy, and Bosentan is substrate of CYP2C9 and 3A4 and cannot be given with cyclosporine or glyburide

39
Q

PDE-5 inhibitors MOA

A

inhibit degradation of cGMP via PDE-5 resulting in inc levels of vasodilatory NO

40
Q

Sildinafil vs Tadalafil

A

Sildinafil approved for 2-3, DI with CYP2C9 and 3A4, Tadalafil approved for 2-3, DI with CYP3A4 and renal hepatic adjust

41
Q

Functional Class 2 treatment

A

Ambrisentan, Bosentan, Sildinafil and Tadalafil

42
Q

Functional Class 3 treatment

A

Ambrisentan, Bosentan, Epoprostenol, Treprostinil, iloprost, sildinafil, tadalafil

43
Q

Functional Class 4 treatment

A

Epoprostenol, Treprostinil, Iloprost

44
Q

If single therapy doesn’t work

A

can combine classes, but then will need trial septostomy and lung ransplant