Baker/Parks: Pulmonary Vascular Pathology Flashcards

1
Q

T/F: Blood clots in the pulmonary arterial system are almost always embolic

A

True!!!

**95% of blood clots in pulmonary arterial system are from DVT

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2
Q

T/F: DVT = PE

A

false, not all DVTs result in a PE

however, PE = DVT in 95% of cases

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3
Q

T/F: Pulmonary embolisms cause significant increases in morbidity and mortality

A

True

1/2 of people with PE have long term complications
1/3 of PE recur over 10 yrs
10-30% die within one month
25% die of sudden death

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4
Q

3 risk factors for pulmonary embolism?

A
  1. endothelial injury (trauma, vasculitis, HTN)
  2. stasis of blood flow (immobility, venous compression)
  3. hypercoagulable (genetic predisposition, cancer, pregnancy)
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5
Q

Two main issues with PEs?

A
  1. respiratory compromise (V/Q mismatch - ventilation does not match tissue perfusion)
  2. hemodynamic compromise - lack of blood flow to lungs
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6
Q

What does the severity of symptoms and PE syndrome depend on?

A

size of PE

**large emboli can cause complete loss of blood flow, leading to hypoxemia, right heart failure and even instantaneous death

**smaller emboli travel deeper into the periphery and are less likely to cause death

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7
Q

This is a hemorrhagic lesion that occurs in 10% of cases of PE
Usu only occurs when underlying cardiopulmonary conditions are present (disrupts the ability of the bronchial arteries to compensate)
Typically occurs in lower lobes

A

pulmonary infarction

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8
Q

Clinical features of PE?

A
tachycardia **most common
chest pain
tachypnea
dyspnea
hypoxemia
cough/fever
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9
Q

3 EKG findings in PE?

A

large S wave in lead 1
pathologic q wave in lead 3
inverted T wave in lead 3

**S1Q3T3

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10
Q

Two imaging modalities that can be used to diagnose PE?

A

chest x-ray: can show wedge-shaped infarcts or V/Q mismatch

CT scan: can show vessels with decreased blood flow if you use contrast

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11
Q

What is normal pulmonary arterial pressure?

A

15-30/4-12

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12
Q

sustained elevation of mean pulmonary arterial pressure to more than 25 mm Hg at rest or to more than 30 mm Hg with exercise

A

pulmonary hypertension

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13
Q

Two types of pulmonary hypertension?

A
primary = idiopathic
secondary = many causes
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14
Q

What will you see histologically in pulmonary hypertension?

A

medial hypertrophy of muscular and elastic arteries
plexiform lesions (in long standing HTN)
pulmonary artery atheromas

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15
Q

What are the 5 clinical classifications of pulmonary arterial hypertension?

A
  1. PAH (primary)
  2. PH with left heart disease
  3. PH associated with lung disease
  4. PH due to chronic thrombotic/embolic disease
  5. Misc PH
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16
Q

Things that can cause secondary pulmonary arterial hypertension?

A

anything that increases pressure!

heart disease, chronic lung disease, obstructive sleep apnea

17
Q

T/F: some patients can have multiple pulmonary emboli

A

True!

**multiple emboli can occlude vessels and lead to pulmonary hypertension

18
Q

What can cause primary pulmonary hypertension?

A

still increased pressure

…but this time due to a mutation in BMPR2 which leads to a proliferation of vascular smooth muscle cells

may be due to environmental factors, too?

19
Q

Who gets pulmonary arterial hypertension?

A

females age 20-40

20
Q

Symptoms of PAH?

A

early on: dyspnea, fatigue, anginal chest pain

later: respiratory distress, cyanosis, RVH, cor pulmonale

**usu die within 2-5 yrs of diagnosis

21
Q

How to treat pulmonary hypertension?

A
O2
calcium channel blockers
digoxin
diuretics
PDE5 inhibitors (Viagra)
inhaled NO
lung transplant
22
Q

Autoimmune disorder – autoantibodies against the basement membrane in alveoli and glomeruli - focal alveolar wall necrosis with intra alveolar hemorrhages
Hemorrhagic interstitial pneumonitis
Rapidly Progressive Glomerulonephritis (RPGN)

A

Goodpasture Syndrome

23
Q

Who gets Goodpasture syndrome?

A

males > females

teens to 20s

24
Q

Diagnostic linear Ig and complement deposits in basement membrane

A

Goodpasture syndrome

25
Clinical features of Goodpasture syndrome?
initial presentation with hemoptysis x-ray = focal pulmonary consolidations renal manifestations follow
26
How to treat Goodpasure syndrome?
Renal replacement therapy (dialysis) for severe AKI Plasmapheresis to remove circulating anti-BM antibodies immunosuppression
27
Intermittent, diffuse alveolar hemorrhage Usually young children Insidious onset of productive cough, hemoptysis, anemia and weight loss Very similar to Goodpasture syndrome, but no anti-BM antibodies Good response to immunosuppression
Idiopathic pulmonary hemosiderosis
28
``` Necrotizing vasculitis Acute necrotizing granulomas Necrotizing or granulomatous vasculitis Renal disease with focal necrotizing, GN Presents with hemoptysis Often diagnosed by transbronchial biopsy ```
Wegener granulomatosis
29
Engorged alveolar capillaries | Intra-alveolar pink precipitate
cardiogenic pulmonary edema
30
What type of cells are seen in cardiogenic pulmonary edema?
"heart failure cells" | hemosiderin-laden macrophages from alveolar microhemorrhages
31
Clinical signs of cardiogenic pulmonary edema
SOB dyspnea on exertion orthopnea crackles on auscultation
32
Where does fluid usu accumulate first in pulmonary edema?
in lower lobes
33
What is one cause of noncardiogenic pulmonary edema?
acute lung injury/ARDS
34
What will you see histologically with noncardiogenic pulmonary edema?
diffuse alveolar damage
35
List four things that can cause acute lung injury, or ARDS?
sepsis diffuse pulmonary infections gastric aspiration mechanical trauma, including head injuries
36
``` What is this? Interstitial edema Intra-alveolar edema Inflammation Fibrin deposition Hyaline membranes Fibrin rich edema fluid mixed with remnants of necrotic epithelia cells ```
diffuse alveolar damage
37
What is the pathogenesis behind ARDS?
systemic/local insult endothelial/epithelial damage: increased vascular permeability, alveolar flooding, reduced diffusion capacity, microthrombi imbalance b/w pro and anti-inflammatory mediators
38
Clinical features of ARDS?
``` rapid onset profound dyspnea tachypnea severe cyanosis respiratory failure diffuse bilateral pulmonary infiltrates very sick ```