Pulmonary HTN

Question 1

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

Group 1
Group 2
Group 3
group 4
group 5

Answer 1

group 1

Question 2

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

Answer 2

group 2

Question 3

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

Answer 3

group 3

Question 4

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

Answer 4

group 4

Question 5

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

Answer 5

group 5

Question 6

Is PAH more common in men or women?

Answer 6

women

Question 7

Mean age of onset of PAH?

Answer 7

37

now more common in 50-65 year olds

Question 8

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

Answer 8

20

Question 9

What -nib drug can cause PAH?

Answer 9

dasatinib

Question 10

__ ventricular hypertrophy and failure can lead to PAH

a. right
b. left

Answer 10

a. right

Question 11

Which type of of echocardiography is used to diagnose PAH?

a. transthoracic
b. esophageal

Answer 11

a. trans thoracic

Question 12

Diagnosis of PAH:

What class is no limitation of usual physical activity?

Answer 12

class I

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

Question 13

Diagnosis of PAH:

Which class mild limitation of physical activity?

Answer 13

class II

Question 14

Diagnosis of PAH:

Which class has marked limitation of physical activity?

Answer 14

class III

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

Question 15

diagnosis of PAH:

What class is unable to perform any physical activity ?

Answer 15

class IV

Question 16

What is done first for diagnosis of PAH?

Answer 16

echocardiography

transthoracic echocardiograph (TTE)

Question 17

Mean pulmonary artery pressure can be estimated using TTE.

a. true
b. false

Answer 17

a. true

Question 18

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

Answer 18

25, 15, 3

Question 19

3 drugs given to assess response in mPAP?

Answer 19

nitric oxide
epoprostenol
adenosine

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

Question 20

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

a. true
b. false

Answer 20

b. false

should NOT be used

Question 21

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

a. true
b. false

Answer 21

a. true

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

Question 22

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

Answer 22

I, II

Question 23

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

a. PGI3
b. PGI2

Answer 23

b. PGI2

Question 24

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

Answer 24

3-5

Question 25

Epoprostenol initiation must be done in a hospital a. true b. false

Answer 25

a. true started on low dose goal dose is 10-15ng/kg/min

Question 26

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

Answer 26

epoprostenol

Question 27

Which epoprostenol brand is the original formulation and requires ice packs, and has low stability? a. Flolan b. Veletri

Answer 27

a. Flolan

Question 28

Which epoprostenol brand is more basic and has greater room temp stability? a. Flolan b. Veletri

Answer 28

b. Veletri

Question 29

Avoid concomitant __ administration with epoprostenol

Answer 29

nitrate risk of severe refractory hypotension

Question 30

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

Answer 30

III also WHO functional class IV

Question 31

Which prostacyclin derivative only had inhalation route for administration? a. epoprostenol b. iloprost c. treprostinil

Answer 31

b. iloprost

Question 32

Which has longer half life, epoprostenol or treprostinil?

Answer 32

treprostinil

Question 33

Max dose of inhaled treprostinil?

Answer 33

9 breaths four times daily

Question 34

Treprostinil should be taken on an empty stomach a. true b. false

Answer 34

b. false must be taken with food to improve absorption

Question 35

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

Answer 35

treprostinil thought to be related to pH of diluent

Question 36

Who should be started on treprostinil?

Answer 36

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

Question 37

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

Answer 37

5

Question 38

___ requires 10 to 15 minutes per inhalation a. iloprost b. treprostinil

Answer 38

a. iloprost

Question 39

__ takes less time per inhalation but is more complicated to prepare a. iloprost b. treprostinil

Answer 39

b. treprostinil

Question 40

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

Answer 40

epoprostenol

Question 41

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

Answer 41

bosentan

Question 42

Bosentan targets the _ receptors a. ETA b. ETb c. both

Answer 42

c. both

Question 43

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

Answer 43

3, 5 may be reintroduced when LFTs return to normal but requires close monitoring

Question 44

Bosentan is pregnancy category X a. true b. false

Answer 44

a. true

Question 45

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

Answer 45

II, III use earlier than epoprostenel may decrease hospitalizations related to PAH in the short term

Question 46

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

Answer 46

ambrisentanLEAP highly recommends two forms of birth control

Question 47

The ERA ambrisentan is started at __ mg once daily

Answer 47

5

Question 48

The ERA ambrisentan targets __ receptor a. ETa b. ETb c. both

Answer 48

a. ETa

Question 49

Transaminitis is very common with ambrisentan a. true b. false

Answer 49

b. false very rare with ambrisentan anemia and edema are still common with ambrisentan

Question 50

The ERA macitentan has 50 fold increased selectivity fo ___ a. ETa b. ETb

Answer 50

a. ETa targets both Eta and ETb but way more selective for b

Question 51

___ is part of the Obsumit REMs program due to teratogenicity a. ambrisentan b. macitentan c. bosentan

Answer 51

b. macitentan monthly pregnancy tests one females are required to register

Question 52

Macitentan has same side effect profile as ___

Answer 52

ambrisentan transaminitis is very rare

Question 53

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

Answer 53

PDE5

Question 54

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

Answer 54

bosentan b/c of CYP3A4

Question 55

riociguat drug class?

Answer 55

soluble cGMP stimulator dose titration limited by hypotension

Question 56

selexipag drug class?

Answer 56

prostacyclin receptor agonist

Question 57

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

Answer 57

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

Question 58

The prostacyclin receptor agonist selexipag is not recommended for use in the guidelines for PAH. a. true b. false

Answer 58

a. true deemed clinically non-significant

Question 59

3 prostacyclin derivatives:

Answer 59

epoprostenol treprostinil ilprost

Question 60

3 ERAs: BAM

Answer 60

bosentan ambrisentan macitentan

Question 61

1 soluble cGMP stimulator:

Answer 61

riociguat

Question 62

1 PRA:

Answer 62

selexipag