08.25 - Pulmonary Hypertension (Muthiah) - Questions Flashcards Preview

Pulmonology > 08.25 - Pulmonary Hypertension (Muthiah) - Questions > Flashcards

Flashcards in 08.25 - Pulmonary Hypertension (Muthiah) - Questions Deck (62):
1

Test critical to confirming and quantifying PAH

Right Heart Catheterization

2

Commonest cause of pulmonary HTN

Pulmonary Venous HTN (post capillary)

3

Definition of PAH

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

4

Alveolar hypoxemia causes what remodeling change

Vasoconstriction --> Chronic thickens vessels

5

Group 1 PAH

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

6

Group 4 PAH

Chronic Thromboembolic

7

Group 5 PAH

Multifactorial etiology

8

Most important vasoconstrictor

Endothelin 1

9

Prostacyclin vs TXA2

Anti-coagulant vs Coagulant

10

Which Collagen Vascular Disease is most often associated with PAH

Systemic Sclerosis

11

Importance of Raynaud's

Conditions with Raynauds often have PAH

12

Thrombocytosis and PAH

Increased 5-HT

13

10% of what hemoglobinopathy is associated with PAH

Sickle Cell - Free Hb and Iron implicated

14

Gene and protein to remember for Familial PPH

PPH1 --> BMPR2

15

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

Stimulants, such as Fen-Phen, Cocaine, etc

16

TXA and Prostacyclin in PPH

Increased TXA, Decr Prostacyclin

17

Nitric Oxide promotes vasodilation and

inhibits SM growth

18

Endothelin 1 in PAH (and 5-HT)

Vasoconstrictor and Mitogen for SM cells - Localises in PAH

19

Vasoconstrictor and Mitogen for SM cells - Localises in PAH

Endothelin 1 in PAH

20

Role of Serotonin in PAH

Vasoconstriction and SM Cell proliferation; Plasma concentrations higher in PAH

21

Role of V-gated K Channels in PAH

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

22

Why is intracellular Ca2+ incr in PAH

Low v-gated K channels

23

Appetite suppressant drugs may generate PAH by

blocking v-gated K channels

24

Role of appetite suppressant drugs

May generate PAH by blocking v-gated K channels

25

Mean age at dx for PPH

36

26

Good screening test for PPH

Echocardiography

27

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

Pulmonary Capillary Wedge Pressure

28

Relevance of Pulmonary Capillary Wedge Pressure

Proportional to LAP and LVEDP

29

Prognosis and percent of patients that are "vasoreactive"

5-6%; Good prognosis

30

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

Nitric Oxide

31

PFT findings in PAH

Normal FEV1, FVC, TLC; Reduced DLCO

32

PFT findings in Emphysema

Reduced FEV1/FVC; Incr TLC; Reduced DLCO

33

Reduced FEV1/FVC; Incr TLC; Reduced DLCO

PFT findings in Emphysema

34

Reduced FVC, TLC, DLCO

Interstitial Lung Disease

35

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

Reduced FVC, TLC, DLCO

36

PFT findings in PE

Normal FEV1, FVC, TLC; Reduced DLCO

37

PFT findings in Anemia

Normal FEV1, FVC, TLC; Reduced DLCO

38

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

PAH, PE, Anemia

39

Drug for PAH Stage 4

Epoprostenol (Flolan)

40

How does Pulmonary Artery Pressure change through PAH progression

Increases, Levels off, Decreases as heart fails

41

How does CO change throughout PAH progression

Constant, Decreases, Drops as heart fails

42

How does PVR change throughout PAH progression

Constantly increases

43

EKG findings in PAH

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

44

RV String Pattern

ST depression with U wave inversion

45

What defines a "response"?

>20% reduction in PAP and PVR

46

Fall in PAP in response to inhaled NO indicates

Patient will also respond to CCB's

47

MOA of Riocuguat

Incr cGMP by stimulating Guanylate Cyclase

48

When is Epoprostenol used

Functional class 4 patients

49

3 major effects of Epoprostenol

Vasodilation; Inhibit platelet aggregation and SM proliferation (unique)

50

Which drug can reverse the remodeling in PAH

Epoprostenol

51

Which PPH patients can benefit from Epoprostenol

Both responders and non-responders

52

How does Epoprostenol cause vasodilation

Incr intracellular cAMP

53

3 side effects of Epoprostenol

Jaw pain, Diarrhea, Arthralgias

54

2 adverse effects of Bosentan

Elevated aminotransferase; Teratogenic

55

Anticoagulant to use in PAH

Warfarin

56

Mean survival for PAH

3 years from dx

57

2 proven therapies to improve survival in PAH

Epoprostenol, Bosentan, Maybe Anticoagulation (warfarin)

58

MOA of Bosentan

Endothelin Receptor Antagonist

59

MOA of Macitentan

Endothelin Receptor Antagonist

60

MOA of Sildenafil/Tadalafil

PDE 5 inhibitor

61

MOA of Epoprostenol

Prostacyclin Analog

62

MOA of Ilopros

Prostacyclin Analog