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

08.25 - Pulmonary Hypertension (Muthiah) - Questions Flashcards

1
Q

Test critical to confirming and quantifying PAH

A

Right Heart Catheterization

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2
Q

Commonest cause of pulmonary HTN

A

Pulmonary Venous HTN (post capillary)

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3
Q

Definition of PAH

A

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

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4
Q

Alveolar hypoxemia causes what remodeling change

A

Vasoconstriction –> Chronic thickens vessels

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5
Q

Group 1 PAH

A

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

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6
Q

Group 4 PAH

A

Chronic Thromboembolic

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7
Q

Group 5 PAH

A

Multifactorial etiology

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8
Q

Most important vasoconstrictor

A

Endothelin 1

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9
Q

Prostacyclin vs TXA2

A

Anti-coagulant vs Coagulant

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10
Q

Which Collagen Vascular Disease is most often associated with PAH

A

Systemic Sclerosis

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11
Q

Importance of Raynaud’s

A

Conditions with Raynauds often have PAH

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12
Q

Thrombocytosis and PAH

A

Increased 5-HT

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13
Q

10% of what hemoglobinopathy is associated with PAH

A

Sickle Cell - Free Hb and Iron implicated

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14
Q

Gene and protein to remember for Familial PPH

A

PPH1 –> BMPR2

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15
Q

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

A

Stimulants, such as Fen-Phen, Cocaine, etc

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16
Q

TXA and Prostacyclin in PPH

A

Increased TXA, Decr Prostacyclin

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17
Q

Nitric Oxide promotes vasodilation and

A

inhibits SM growth

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18
Q

Endothelin 1 in PAH (and 5-HT)

A

Vasoconstrictor and Mitogen for SM cells - Localises in PAH

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19
Q

Vasoconstrictor and Mitogen for SM cells - Localises in PAH

A

Endothelin 1 in PAH

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20
Q

Role of Serotonin in PAH

A

Vasoconstriction and SM Cell proliferation; Plasma concentrations higher in PAH

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21
Q

Role of V-gated K Channels in PAH

A

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

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22
Q

Why is intracellular Ca2+ incr in PAH

A

Low v-gated K channels

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23
Q

Appetite suppressant drugs may generate PAH by

A

blocking v-gated K channels

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24
Q

Role of appetite suppressant drugs

A

May generate PAH by blocking v-gated K channels

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25
Q

Mean age at dx for PPH

A

36

26
Q

Good screening test for PPH

A

Echocardiography

27
Q

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

A

Pulmonary Capillary Wedge Pressure

28
Q

Relevance of Pulmonary Capillary Wedge Pressure

A

Proportional to LAP and LVEDP

29
Q

Prognosis and percent of patients that are “vasoreactive”

A

5-6%; Good prognosis

30
Q

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

A

Nitric Oxide

31
Q

PFT findings in PAH

A

Normal FEV1, FVC, TLC; Reduced DLCO

32
Q

PFT findings in Emphysema

A

Reduced FEV1/FVC; Incr TLC; Reduced DLCO

33
Q

Reduced FEV1/FVC; Incr TLC; Reduced DLCO

A

PFT findings in Emphysema

34
Q

Reduced FVC, TLC, DLCO

A

Interstitial Lung Disease

35
Q

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

A

Reduced FVC, TLC, DLCO

36
Q

PFT findings in PE

A

Normal FEV1, FVC, TLC; Reduced DLCO

37
Q

PFT findings in Anemia

A

Normal FEV1, FVC, TLC; Reduced DLCO

38
Q

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

A

PAH, PE, Anemia

39
Q

Drug for PAH Stage 4

A

Epoprostenol (Flolan)

40
Q

How does Pulmonary Artery Pressure change through PAH progression

A

Increases, Levels off, Decreases as heart fails

41
Q

How does CO change throughout PAH progression

A

Constant, Decreases, Drops as heart fails

42
Q

How does PVR change throughout PAH progression

A

Constantly increases

43
Q

EKG findings in PAH

A

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

44
Q

RV String Pattern

A

ST depression with U wave inversion

45
Q

What defines a “response”?

A

> 20% reduction in PAP and PVR

46
Q

Fall in PAP in response to inhaled NO indicates

A

Patient will also respond to CCB’s

47
Q

MOA of Riocuguat

A

Incr cGMP by stimulating Guanylate Cyclase

48
Q

When is Epoprostenol used

A

Functional class 4 patients

49
Q

3 major effects of Epoprostenol

A

Vasodilation; Inhibit platelet aggregation and SM proliferation (unique)

50
Q

Which drug can reverse the remodeling in PAH

A

Epoprostenol

51
Q

Which PPH patients can benefit from Epoprostenol

A

Both responders and non-responders

52
Q

How does Epoprostenol cause vasodilation

A

Incr intracellular cAMP

53
Q

3 side effects of Epoprostenol

A

Jaw pain, Diarrhea, Arthralgias

54
Q

2 adverse effects of Bosentan

A

Elevated aminotransferase; Teratogenic

55
Q

Anticoagulant to use in PAH

A

Warfarin

56
Q

Mean survival for PAH

A

3 years from dx

57
Q

2 proven therapies to improve survival in PAH

A

Epoprostenol, Bosentan, Maybe Anticoagulation (warfarin)

58
Q

MOA of Bosentan

A

Endothelin Receptor Antagonist

59
Q

MOA of Macitentan

A

Endothelin Receptor Antagonist

60
Q

MOA of Sildenafil/Tadalafil

A

PDE 5 inhibitor

61
Q

MOA of Epoprostenol

A

Prostacyclin Analog

62
Q

MOA of Ilopros

A

Prostacyclin Analog

Pulmonology (31 decks)

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