B4.067 Pulmonary Vascular Disease Flashcards

1
Q

3 major diseases of the pulmonary vessels

A

pulmonary embolism
pulmonary hypertension
pulmonary edema

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2
Q

normal RA pressure

A

0-8 mmHg

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3
Q

normal RV pressure

A

20-30/0-8 mmHg

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4
Q

normal pulm artery pressure

A

20-30/8-15 mmHg

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5
Q

normal pulmonary artery wedge pressure

A

8-12 mmHg

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6
Q

how do you get a wedge pressure?

A

extend catheter through pulm artery until it completely occludes a vessel and the only pressure is the backpressure

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7
Q

how do you calculate pulmonary vascular resistance?

A

(mean pulm artery pressure - LA pressure) / pulm blood flow

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8
Q

what are the 5 primary classes of pulmonary hypertension

A
  1. pulmonary artery hypertension
  2. left heart disease
  3. PH with respiratory disease or hypoxia
  4. chronic thromboembolic disease
  5. unclear/multifactorial
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9
Q

what does the new classification system emphasize?

A

similar pathologic findings, similar hemodynamic characteristics, and similar management

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10
Q

what is pulmonary arterial hypertension (PAH)?

A

sustained elevation of mean pulm arterial pressure of > 25 mmHg
mean pulmonary capillary wedge pressure and/or mean LV end diastolic pressure <15 mmHg

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11
Q

what is the multiple hit hypothesis of the pathogenesis of PAH

A

primary genetic background + modifier genes + environmental trigger = PAH

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12
Q

what are some examples of environmental triggers of PAH

A

meth

scleroderma

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13
Q

discuss the pathology of PAH

A

pulm artery remodeling:

  • intimal fibrosis
  • medial hypertrophy
  • adventitial proliferation
  • luminal obliteration
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14
Q

what are some effects of PAH

A
RV hypertrophy
increased central venous pressure
peripheral edema
JVD
SOB
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15
Q

what 3 pathways may target PAH

A

endothelin
NO
prostacyclin

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16
Q

discuss prostacyclin

A
activity through cAMP
vasodilator
inhibits proliferation of vascular smooth muscle
decreases platelet aggregation
decreased prostacyclin synthase in PAH
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17
Q

discuss endothelin 1

A

increases intravascular volume and CO by being a potent vasoconstrictor
inhibiting this may help with PAH

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18
Q

what are goals of PAH therapy

A

dilate blood vessels
help heart
prevent blood clots
decrease scarring/blocked vessels

19
Q

what are some options for PAH supportive therapy

A

oxygen
Coumadin
diuretics
treatment of underlying/ co-existing disease

20
Q

spectrum of treatment options for PAH from least to most intensive

A
nothing
oral meds
nebulized meds
continuously infused meds
gene therapy
lung transplant
21
Q

classes of drugs that help with PAH

A

prostacyclin
prostacyclin analogues
phosphodiesterase inhibitors (increases NO concentration)
endothelin-receptor antagonists

22
Q

how do thrombi form?

A

blood stasis
hyper-coagulable states
vessel wall abnormalities

23
Q

where do pulmonary emboli originate?

A

deep veins of the lower extremities or pelvis
upper extremity veins (catheters)
right heart chamber (a fib)
superior vena cava

24
Q

what is the effect of a pulmonary embolus?

A
decreases or total cessation of pulm blood flow to the affected distal zone
increased PVR (50% occlusion necessary)
25
Q

what happens when pulm blood flow decreases?

A

physiologic dead space increased
bronchoconstriction
surfactant production decreases-resulting in atelectasis
arterial hypoxemia

26
Q

what happens when PVR increases after 50% occlusion occurs?

A

when mean PAP reaches > 40 mmHg, RV will fail and collapse occurs

27
Q

what causes death from PE?

A

cardio collapse, not resp failure

28
Q

characterize resolution of PEs

A

resolution occurs rapidly with only a small percentage suffering permanent perfusion defects

29
Q

what are clinical signs of PE

A
pain/swelling of extremities (DVT)
dyspnea (sudden onset)
pleuritic chest pain
cough
apprehension
hemoptysis
tachycardia, tachypnea, hypoxia
30
Q

how do you diagnose DVT

A

ultrasound

31
Q

how do you diagnose PE

A

CT chest

32
Q

how do you prevent PE

A

most immobilized hospitalized patients need prophylaxis for DVT

  • pharmacological anticoagulants
  • mechanical: pneumatic calf compression
33
Q

how do you treat an acute DVT or PE?

A

anticoagulation

heparin/warfarin/NOACS

34
Q

what are supportive therapies for PE?

A

oxygen to treat hypoxemia
fluid and vasopressors for hypotension and shock
thrombolytics only when shock/cardiac arrest
embolectomy

35
Q

what are 2 types of pulm edema

A

cardiogenic

non-cardiogenic

36
Q

what is the cause of cardiogenic pulm edema

A

pressure related phenomenon

LV problems, left sided valve problems, pulm vein obstructions

37
Q

what is the cause of noncaridogenic pulm edema

A

leaky capillaries

ARDS, HAPE, neurogenic, opiate overdose

38
Q

how do lungs typically protect themselves from excessive accumulation of fluid into interstitial spaces?

A

lymphatic drainage into SVC aided by changes in intrathoracic pressure with normal respiration
loose connective tissue located along peribronchovascular space and extending to the level of the bronchiole is capable of storing 2x the normal fluid content of the lungs
gel like matrix of the lung is capable of absorbing additional fluid without affecting interstitial pressures

39
Q

pathophysiology of pulm edema

A
decreased lung compliance
decreased lung volumes
increased airway resistance
increased work of breathing
V/Q mismatch
increased (A-a)O2 gradient
hypoxemia
40
Q

clinical findings of mild pulm edema

A

dyspnea on exertion
orthopnea
few rales in bases
variable peripheral edema

41
Q

clinical findings of acute/severe pulm edema

A
productive cough of frothy blood tinged sputum
tachypnea
apprehensive
peripheral extremities cool, clammy, cyanotic
rales, wheezing throughout
engorged neck veins
tachycardia
cardiac enlargement
42
Q

how to manage cardiogenic edema

A

improve cardiac function (afterload reduction, inotropes)
eliminate excess fluid
oxygen/ventilator support
advanced therapies : ECMO, LVAD

43
Q

how to manage non-cardiogenic edema

A

oxygen/respiratory support
ICU care
advanced support
treat underlying disease