08.25 - Pulmonary Hypertension (Muthiah) - Questions

Question 1

Test critical to confirming and quantifying PAH

Answer 1

Right Heart Catheterization

Question 2

Commonest cause of pulmonary HTN

Answer 2

Pulmonary Venous HTN (post capillary)

Question 3

Definition of PAH

Answer 3

Mean PAP > 25mmHg at rest (Mean PCWP&LVEDP <15)

Question 4

Alveolar hypoxemia causes what remodeling change

Answer 4

Vasoconstriction –> Chronic thickens vessels

Question 5

Group 1 PAH

Answer 5

iPAH, Heritable (BMPR2), HIV-related, CTDz

Question 6

Group 4 PAH

Answer 6

Chronic Thromboembolic

Question 7

Group 5 PAH

Answer 7

Multifactorial etiology

Question 8

Most important vasoconstrictor

Answer 8

Endothelin 1

Question 9

Prostacyclin vs TXA2

Answer 9

Anti-coagulant vs Coagulant

Question 10

Which Collagen Vascular Disease is most often associated with PAH

Answer 10

Systemic Sclerosis

Question 11

Importance of Raynaud’s

Answer 11

Conditions with Raynauds often have PAH

Question 12

Thrombocytosis and PAH

Answer 12

Increased 5-HT

Question 13

10% of what hemoglobinopathy is associated with PAH

Answer 13

Sickle Cell - Free Hb and Iron implicated

Question 14

Gene and protein to remember for Familial PPH

Answer 14

PPH1 –> BMPR2

Question 15

History of what emphasized class of medications is important to obtain in patient with suspected PAH?

Answer 15

Stimulants, such as Fen-Phen, Cocaine, etc

Question 16

TXA and Prostacyclin in PPH

Answer 16

Increased TXA, Decr Prostacyclin

Question 17

Nitric Oxide promotes vasodilation and

Answer 17

inhibits SM growth

Question 18

Endothelin 1 in PAH (and 5-HT)

Answer 18

Vasoconstrictor and Mitogen for SM cells - Localises in PAH

Question 19

Vasoconstrictor and Mitogen for SM cells - Localises in PAH

Answer 19

Endothelin 1 in PAH

Question 20

Role of Serotonin in PAH

Answer 20

Vasoconstriction and SM Cell proliferation; Plasma concentrations higher in PAH

Question 21

Role of V-gated K Channels in PAH

Answer 21

Lower mRNA in PAH - Low channel current - Incr in intracellular Ca2+ - Vasoconstriction

Question 22

Why is intracellular Ca2+ incr in PAH

Answer 22

Low v-gated K channels

Question 23

Appetite suppressant drugs may generate PAH by

Answer 23

blocking v-gated K channels

Question 24

Role of appetite suppressant drugs

Answer 24

May generate PAH by blocking v-gated K channels

Question 25

Mean age at dx for PPH

Answer 25

36

Question 26

Good screening test for PPH

Answer 26

Echocardiography

Question 27

In an RHC report, the parameter that distinguishes PAH from PVH:

Answer 27

Pulmonary Capillary Wedge Pressure

Question 28

Relevance of Pulmonary Capillary Wedge Pressure

Answer 28

Proportional to LAP and LVEDP

Question 29

Prognosis and percent of patients that are “vasoreactive”

Answer 29

5-6%; Good prognosis

Question 30

During RHC, which is the agent of choice for vasoreactivity testing?

Answer 30

Nitric Oxide

Question 31

PFT findings in PAH

Answer 31

Normal FEV1, FVC, TLC; Reduced DLCO

Question 32

PFT findings in Emphysema

Answer 32

Reduced FEV1/FVC; Incr TLC; Reduced DLCO

Question 33

Reduced FEV1/FVC; Incr TLC; Reduced DLCO

Answer 33

PFT findings in Emphysema

Question 34

Reduced FVC, TLC, DLCO

Answer 34

Interstitial Lung Disease

Question 35

PFT findings in Interstitial Lung Disease (FVC, TLC, DLCO)

Answer 35

Reduced FVC, TLC, DLCO

Question 36

PFT findings in PE

Answer 36

Normal FEV1, FVC, TLC; Reduced DLCO

Question 37

PFT findings in Anemia

Answer 37

Normal FEV1, FVC, TLC; Reduced DLCO

Question 38

Normal FEV1, FVC, TLC; Reduced DLCO (name 3 conditions)

Answer 38

PAH, PE, Anemia

Question 39

Drug for PAH Stage 4

Answer 39

Epoprostenol (Flolan)

Question 40

How does Pulmonary Artery Pressure change through PAH progression

Answer 40

Increases, Levels off, Decreases as heart fails

Question 41

How does CO change throughout PAH progression

Answer 41

Constant, Decreases, Drops as heart fails

Question 42

How does PVR change throughout PAH progression

Answer 42

Constantly increases

Question 43

EKG findings in PAH

Answer 43

Tall p wave in 2; Tall R in aVR and V1; RV string pattern

Question 44

RV String Pattern

Answer 44

ST depression with U wave inversion

Question 45

What defines a “response”?

Answer 45

> 20% reduction in PAP and PVR

Question 46

Fall in PAP in response to inhaled NO indicates

Answer 46

Patient will also respond to CCB’s

Question 47

MOA of Riocuguat

Answer 47

Incr cGMP by stimulating Guanylate Cyclase

Question 48

When is Epoprostenol used

Answer 48

Functional class 4 patients

Question 49

3 major effects of Epoprostenol

Answer 49

Vasodilation; Inhibit platelet aggregation and SM proliferation (unique)

Question 50

Which drug can reverse the remodeling in PAH

Answer 50

Epoprostenol

Question 51

Which PPH patients can benefit from Epoprostenol

Answer 51

Both responders and non-responders

Question 52

How does Epoprostenol cause vasodilation

Answer 52

Incr intracellular cAMP

Question 53

3 side effects of Epoprostenol

Answer 53

Jaw pain, Diarrhea, Arthralgias

Question 54

2 adverse effects of Bosentan

Answer 54

Elevated aminotransferase; Teratogenic

Question 55

Anticoagulant to use in PAH

Answer 55

Warfarin

Question 56

Mean survival for PAH

Answer 56

3 years from dx

Question 57

2 proven therapies to improve survival in PAH

Answer 57

Epoprostenol, Bosentan, Maybe Anticoagulation (warfarin)

Question 58

MOA of Bosentan

Answer 58

Endothelin Receptor Antagonist

Question 59

MOA of Macitentan

Answer 59

Endothelin Receptor Antagonist

Question 60

MOA of Sildenafil/Tadalafil

Answer 60

PDE 5 inhibitor

Question 61

MOA of Epoprostenol

Answer 61

Prostacyclin Analog

Question 62

MOA of Ilopros

Answer 62

Prostacyclin Analog