Pulmonary HTN Flashcards

1
Q

What group of pulmonary HTN is caused by pulmonary artery hypertension?

Group 1
Group 2
Group 3
group 4
group 5
A

group 1

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2
Q

What group of pulmonary HTN is caused by left heart disease?

A

group 2

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3
Q

What group of pulmonary HTN is caused by lung diseases and/or hypoxia?

A

group 3

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4
Q

What group of pulmonary HTN is caused by chronic thromboembolic pulmonary HTN?

A

group 4

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5
Q

Which group of pulmonary HTN has unclear and/or multifactorial cause?

A

group 5

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6
Q

Is PAH more common in men or women?

A

women

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7
Q

Mean age of onset of PAH?

A

37

now more common in 50-65 year olds

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8
Q

Pregnant women exposed to SSRIs after __ weeks gestation can develop PAH

A

20

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9
Q

What -nib drug can cause PAH?

A

dasatinib

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10
Q

__ ventricular hypertrophy and failure can lead to PAH

a. right
b. left

A

a. right

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11
Q

Which type of of echocardiography is used to diagnose PAH?

a. transthoracic
b. esophageal

A

a. trans thoracic

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12
Q

Diagnosis of PAH:

What class is no limitation of usual physical activity?

A

class I

ordinary physical activity does not cause increased dyspnea, fatigue, chest pain, of presyncope

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13
Q

Diagnosis of PAH:

Which class mild limitation of physical activity?

A

class II

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14
Q

Diagnosis of PAH:

Which class has marked limitation of physical activity?

A

class III

no discomfort at rest, but LESS than normal ;physical activity causes increased dyspnea, fatigue, chest pain, or presyncope

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15
Q

diagnosis of PAH:

What class is unable to perform any physical activity ?

A

class IV

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16
Q

What is done first for diagnosis of PAH?

A

echocardiography

transthoracic echocardiograph (TTE)

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17
Q

Mean pulmonary artery pressure can be estimated using TTE.

a. true
b. false

A

a. true

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18
Q

Diagnosis criteria is mean pulmonary artery pressure of >= __mmHg at rest PLUS PCWP <= __ mmHg PLUS pulmonary vascular resistance > __ wood units

A

25, 15, 3

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19
Q

3 drugs given to assess response in mPAP?

A

nitric oxide
epoprostenol
adenosine

if reduced by at least 10mmHg to <= 40mmHg it is considered positive

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20
Q

CCBs should be used in pts with a negative pulmonary vasoreactivity test

a. true
b. false

A

b. false

should NOT be used

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21
Q

CCBs for PAH should only be used for pts with a positive pulmonary vasoreactivity test

a. true
b. false

A

a. true

nifedipine 120-140mg
diltiazem 240-720mg
amlodipine 20mg

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22
Q

With CCBs if pt does not improve to functional class __ or __ additional or alternative PAH therapy should be started

A

I, II

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23
Q

The prostacyclin derivatives epoprostenol, treprostinil, and iloprost are synthetic analogs of __

a. PGI3
b. PGI2

A

b. PGI2

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24
Q

The prostacyclin derivative epoprostenol has a very short half life of ___ minutes

A

3-5

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25
Q

Epoprostenol initiation must be done in a hospital

a. true
b. false

A

a. true

started on low dose

goal dose is 10-15ng/kg/min

26
Q

The patient must have a backup supply of which prostacyclin derivative?

A

epoprostenol

27
Q

Which epoprostenol brand is the original formulation and requires ice packs, and has low stability?

a. Flolan
b. Veletri

A

a. Flolan

28
Q

Which epoprostenol brand is more basic and has greater room temp stability?

a. Flolan
b. Veletri

A

b. Veletri

29
Q

Avoid concomitant __ administration with epoprostenol

A

nitrate

risk of severe refractory hypotension

30
Q

WHO functional class __ with rapid disease progression or poor prognosis should be started on epoprostenol

A

III

also WHO functional class IV

31
Q

Which prostacyclin derivative only had inhalation route for administration?

a. epoprostenol
b. iloprost
c. treprostinil

A

b. iloprost

32
Q

Which has longer half life, epoprostenol or treprostinil?

A

treprostinil

33
Q

Max dose of inhaled treprostinil?

A

9 breaths four times daily

34
Q

Treprostinil should be taken on an empty stomach

a. true
b. false

A

b. false

must be taken with food to improve absorption

35
Q

Which prostacyclin derivative has more cases of bloodstream infections, treprostinil or epoprostenol?

A

treprostinil

thought to be related to pH of diluent

36
Q

Who should be started on treprostinil?

A

class III with rapid disease/poor prognosis and/or class IV

37
Q

The goal for iloprost is to titrate up to __mcg per dose if pt can tolerate

A

5

38
Q

___ requires 10 to 15 minutes per inhalation

a. iloprost
b. treprostinil

A

a. iloprost

39
Q

__ takes less time per inhalation but is more complicated to prepare

a. iloprost
b. treprostinil

A

b. treprostinil

40
Q

Which prostacyclin derivative has shown an improvement in survival compared with historical controls?

A

epoprostenol

41
Q

Which endothelia receptor antagonist (ERA) is part of a distribution program Tracleer access program?

A

bosentan

42
Q

Bosentan targets the _ receptors

a. ETA
b. ETb
c. both

A

c. both

43
Q

Bosentan should be dose reduced or held if AST/ALT is __ to __ times the upper limit of normal

A

3, 5

may be reintroduced when LFTs return to normal but requires close monitoring

44
Q

Bosentan is pregnancy category X

a. true
b. false

A

a. true

45
Q

Bosentan should be started in pts that are WHO functional class __ or __

A

II, III

use earlier than epoprostenel

may decrease hospitalizations related to PAH in the short term

46
Q

Which ERA is part of the Letairis education and access program (LEAP) program due to teratogenicity

A

ambrisentanLEAP highly recommends two forms of birth control

47
Q

The ERA ambrisentan is started at __ mg once daily

A

5

48
Q

The ERA ambrisentan targets __ receptor

a. ETa
b. ETb
c. both

A

a. ETa

49
Q

Transaminitis is very common with ambrisentan

a. true
b. false

A

b. false

very rare with ambrisentan

anemia and edema are still common with ambrisentan

50
Q

The ERA macitentan has 50 fold increased selectivity fo ___

a. ETa
b. ETb

A

a. ETa

targets both Eta and ETb but way more selective for b

51
Q

___ is part of the Obsumit REMs program due to teratogenicity

a. ambrisentan
b. macitentan
c. bosentan

A

b. macitentan

monthly pregnancy tests

one females are required to register

52
Q

Macitentan has same side effect profile as ___

A

ambrisentan

transaminitis is very rare

53
Q

Patients who remain symptomatic on stable doses of a ___ or an inhaled prostanoid should be started on macitentan

A

PDE5

54
Q

There is a 50% decrease in sildenafil concentrations with which ERA?

A

bosentan

b/c of CYP3A4

55
Q

riociguat drug class?

A

soluble cGMP stimulator

dose titration limited by hypotension

56
Q

selexipag drug class?

A

prostacyclin receptor agonist

57
Q

The prostacyclin receptor agonist selexipag has a DDI with what drug?

A

gemfibrozil

gemfibrozil may increase selexipag concentrations by two fold and its metabolite concentrations by 11 fold

58
Q

The prostacyclin receptor agonist selexipag is not recommended for use in the guidelines for PAH.

a. true
b. false

A

a. true

deemed clinically non-significant

59
Q

3 prostacyclin derivatives:

A

epoprostenol
treprostinil
ilprost

60
Q

3 ERAs:

BAM

A

bosentan
ambrisentan
macitentan

61
Q

1 soluble cGMP stimulator:

A

riociguat

62
Q

1 PRA:

A

selexipag