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Flashcards in Pulmonary HTN Deck (60)
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1

What drug is known to cause hemorrhagic cystitis?

cyclophosphamide

2

How is hemorrhagic cystitis prevented with cyclophosphamide?

Mesna

3

When does pulmonary VENOUS HTN occur?

CHF. When the LV fails, stroke volume drops and extra volume and pressure leak back to the pulmonary veins from the left heart

4

What is PAP in PAH?

25+ mm Hg at rest (30+ in exercise)

5

What PCWP (right heart cath required) and LVEDP (left heart catheterization required) is seen in PAH?

less than 15 mm Hg IN BOTH (because there is no pulmonary venous HTN)

Rules out LV dysfunction

6

What is LVEDP is more than 15 mm Hg?

suggests LV failure because of original pulmonary venous HTN

7

What causes primary PAH (iPAH)?

Unknown (absence of a demonstrable cause)

8

What things can cause PAH (these cause 2ndary PAH)?

-parenchymal lung disease

-chronic thromboembolic disease

-LV valve disease

-myocardial disease

-any condition that promotes hypoxic vasoconstriction

9

There are five groups of PAH. What are groups 1-3?

1- PAH (iPAH, PortoPulm, Heritable, BMPR2, CT tissue)

2- PAH due to heart disease

3- PAH due to lung disease

10

What are groups 4-5?

4-CTEPH: Chr Thromboembolic Pulm HTN

5- PAH due to multifactorial etiology

11

When are plexiform lesions seen?

mostly in iPAH and familial PAH

12

What vascular factors are upregulated in PAH?

-Endothelin-1
-TXA-2
-VEGF (overexpressed in plexiform lesions)
-serotonin (constrictor)

13

What exogenous mediators promote vasoconstriction of the pulmonary artery?

-anorexiants
-cocaine

14

What vascular factors are downregulated in PAH?

-NO
-Prostacyclin

15

Which collagen vascular disease is most often associated with PAH?

Systemic sclerosis

16

What are some other pathologies associated with PAH?

-SLE
-MCTD
-RA
-HIV
-SCD
-Thrombocytosis

can get Raynaud's phenomenon

17

What percentage of patients with portal HTN show PAH?

5%

18

Why would PAH been seen in hemoglobinopathies like SCD?

Free iron is released and iron scavenges NO and neutralizes it

19

MOI of Familial PAH

AD (incomplete penetrance)

20

What gene is messed up in familial PAH?

PPH1 on chromosome 2

21

What does defective PPH1 result in?

defective function of the bone morphogenetic protein receptors type II (BMPR2)

22

What do carriers of PPH1 show?

abnormal pulmonary vascular response to exercise

23

What are the effects of NO?

vasodilation and inhibition of smooth muscle growth (nearly absent in PAH)

24

Where is serotonin stored?

platelets mainly

25

What does serotonin promote?

pulmonary vasoconstriction and proliferation of smooth muscle cells

26

T or F. Plasma serotonin concentrations are higher than normal in PAH

T.

27

How is Ca2+ mediated constriction different in PAH?

voltage-gated K+ channels that normally control the release of calcium are defective and you see an INCREASE in intracellular calcium and thus more contraction

28

Mean age of PAH diagnosis

36

29

How does PAH present?

-fatigue
-exertional dyspnea, chest pain and syncope
-edema eventually due to RV failure

30

Signs of PAH?

-OUTWARD (not down and out) shift of the apical impulse
-dyspnea
-widely split S2
-parasternal heave
-palpable P2