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Pulmonary Week 3 > Pulmonary HTN > Flashcards

Pulmonary HTN Flashcards

1
Q

What drug is known to cause hemorrhagic cystitis?

A

cyclophosphamide

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2
Q

How is hemorrhagic cystitis prevented with cyclophosphamide?

A

Mesna

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3
Q

When does pulmonary VENOUS HTN occur?

A

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

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4
Q

What is PAP in PAH?

A

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

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5
Q

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

A

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

Rules out LV dysfunction

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6
Q

What is LVEDP is more than 15 mm Hg?

A

suggests LV failure because of original pulmonary venous HTN

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7
Q

What causes primary PAH (iPAH)?

A

Unknown (absence of a demonstrable cause)

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8
Q

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

A
  • parenchymal lung disease
  • chronic thromboembolic disease
  • LV valve disease
  • myocardial disease
  • any condition that promotes hypoxic vasoconstriction
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9
Q

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

A

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

2- PAH due to heart disease

3- PAH due to lung disease

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10
Q

What are groups 4-5?

A

4-CTEPH: Chr Thromboembolic Pulm HTN

5- PAH due to multifactorial etiology

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11
Q

When are plexiform lesions seen?

A

mostly in iPAH and familial PAH

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12
Q

What vascular factors are upregulated in PAH?

A
  • Endothelin-1
  • TXA-2
  • VEGF (overexpressed in plexiform lesions)
  • serotonin (constrictor)
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13
Q

What exogenous mediators promote vasoconstriction of the pulmonary artery?

A
  • anorexiants

- cocaine

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14
Q

What vascular factors are downregulated in PAH?

A
  • NO

- Prostacyclin

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15
Q

Which collagen vascular disease is most often associated with PAH?

A

Systemic sclerosis

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16
Q

What are some other pathologies associated with PAH?

A
  • SLE
  • MCTD
  • RA
  • HIV
  • SCD
  • Thrombocytosis

can get Raynaud’s phenomenon

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17
Q

What percentage of patients with portal HTN show PAH?

A

5%

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18
Q

Why would PAH been seen in hemoglobinopathies like SCD?

A

Free iron is released and iron scavenges NO and neutralizes it

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19
Q

MOI of Familial PAH

A

AD (incomplete penetrance)

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20
Q

What gene is messed up in familial PAH?

A

PPH1 on chromosome 2

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21
Q

What does defective PPH1 result in?

A

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

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22
Q

What do carriers of PPH1 show?

A

abnormal pulmonary vascular response to exercise

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23
Q

What are the effects of NO?

A

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

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24
Q

Where is serotonin stored?

A

platelets mainly

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25
Q

What does serotonin promote?

A

pulmonary vasoconstriction and proliferation of smooth muscle cells

26
Q

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

A

T.

27
Q

How is Ca2+ mediated constriction different in PAH?

A

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
Q

Mean age of PAH diagnosis

A

36

29
Q

How does PAH present?

A
  • fatigue
  • exertional dyspnea, chest pain and syncope
  • edema eventually due to RV failure
30
Q

Signs of PAH?

A
  • OUTWARD (not down and out) shift of the apical impulse
  • dyspnea
  • widely split S2
  • parasternal heave
  • palpable P2
31
Q

When is a helical CT with contrast indicated?

A

to rule out pulmonary embolism

32
Q

When are High resolution CT scan indicated?

A

to rule out interstitial disease

33
Q

What is the best initial test for PAH?

A

Echocardiography (good screen)

34
Q

In a RHC (right heart catheterization) report, the parameter that distinguishes PAH from PVH is what?

A

The PCWP

35
Q

T or F. Vasoreactive patients have a relatively better prognosis compared to those with vasoreactivity

A

T. This confirms that CCBs are safe (the majority of patients are not vasoreactive)

36
Q

During RHC, what is the best agent for vaso-reactivity testing?

A

NO (inhaled and see immediate acting and short duration)

Inhaled while the catheter is in place

37
Q

What is a positive vasoreaction defined as?

A

20+% reduction in PAP and PVR with increased or unchanged cardiac output and minimally reduced or unchanged systemic blood pressure and SVR

38
Q

Quantified requirements for a positive vasoreactive test

A

10+ mm Hg drop in MPAP

and MPAP should drop to less than 40mm Hg

39
Q

What would an ECG show to indicate PAH?

A

shows evidence of RH enlargement, tricuspid insufficiency, and paradoxical motion of the IVS

40
Q

How is tricuspid insufficiency calculated?

A

4V (velocity) ^2 + RA pressure

41
Q

What are two diseases where PaO2 will decrease with walking/exercise early on?

A
  • pulmonary fibrosis

- PAH

42
Q

What patients are prime for sleep studies?

A

those with BMI 35+ and look at risk for sleep apnea

43
Q

How to confirm PCWP is not high?

A

RH catheterization (this is the confirmatory test for PAH)

44
Q

What are the typical PFT findings in PAH?

A

Normal FEV1, FVC, TLC and REDUCED DLCO

aka ‘pulmonary vascular disease pattern’

45
Q

Which agent should be selected for a patient with PAH, who is SOB at rest (WHO class IV)?

A

Ipoprostenol (only available IV)

46
Q

What pattern on an EKG is typical of PAH?

A

S1Q3T3

47
Q

What is a PGI2 receptor agonist?

A

Selexipag (brand new)

48
Q

What is a drug used to increase cGMP?

A

Riociguat

49
Q

Adjuvant therapy for PAH?

A

oxygen (get hypoxemia at rest sometimes) and

anticoagulation in Group I patients (PPH)

50
Q

Why are patients with PPH at an increased risk of intrapulmonary thrombosis or VTE?

A

sluggish pulmonary blood blood, dilated right heart chambers, venous stasis, and commonly sedentary lifestyles

51
Q

What is the current anticoagulant drug of choice for PAH?

A

Warfarin (Goal: INR of 2)

52
Q

How is the prognosis of PAH?

A

Variable depending on the severity of hemodynamic derangement an the response to vasodilator therapy

mean survival: 3 yrs

53
Q

Which groups of PAH tend to live longer?

A

I and II

54
Q

What are some ECG findings associated with poor outcome in PAH?

A
  • large right atrium
  • presence of pericardial effusion
  • large degree of septal shift during diastole
55
Q

Patients with PAH are at risk for both ____ and ____.

A

progressive right heart failure and sudden cardiac death.

CPR after CV collapse in PAH pts is generally unsuccessful

56
Q

Is edema seen in PAH?

A

Yes (diuretics help) and JVD is seen

57
Q

When to suspect PAH?

A

Suspect PAH in patients with insidious onset, gradually progressive shortness of breath without wheezing

58
Q

What should be done if lung function continues to deteriorate in PAH?

A

refer for lung transplant

59
Q

Best drugs for PAH?

A

Epoprostenol and Bosentan

60
Q

Advantage of Macitentan?

A

less liver toxicity

Pulmonary Week 3 (18 decks)

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