Baker/Parks: Pulmonary Vascular Pathology

Question 1

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

Answer 1

True!!!

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

Question 2

T/F: DVT = PE

Answer 2

false, not all DVTs result in a PE

however, PE = DVT in 95% of cases

Question 3

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

Answer 3

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

Question 4

3 risk factors for pulmonary embolism?

Answer 4

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

Question 5

Two main issues with PEs?

Answer 5

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

Question 6

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

Answer 6

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

Question 7

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

Answer 7

pulmonary infarction

Question 8

Clinical features of PE?

Answer 8

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

Question 9

3 EKG findings in PE?

Answer 9

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

**S1Q3T3

Question 10

Two imaging modalities that can be used to diagnose PE?

Answer 10

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

Question 11

What is normal pulmonary arterial pressure?

Answer 11

15-30/4-12

Question 12

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

Answer 12

pulmonary hypertension

Question 13

Two types of pulmonary hypertension?

Answer 13

primary = idiopathic
secondary = many causes

Question 14

What will you see histologically in pulmonary hypertension?

Answer 14

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

Question 15

What are the 5 clinical classifications of pulmonary arterial hypertension?

Answer 15

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

Question 16

Things that can cause secondary pulmonary arterial hypertension?

Answer 16

anything that increases pressure!

heart disease, chronic lung disease, obstructive sleep apnea

Question 17

T/F: some patients can have multiple pulmonary emboli

Answer 17

True!

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

Question 18

What can cause primary pulmonary hypertension?

Answer 18

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?

Question 19

Who gets pulmonary arterial hypertension?

Answer 19

females age 20-40

Question 20

Symptoms of PAH?

Answer 20

early on: dyspnea, fatigue, anginal chest pain

later: respiratory distress, cyanosis, RVH, cor pulmonale

**usu die within 2-5 yrs of diagnosis

Question 21

How to treat pulmonary hypertension?

Answer 21

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

Question 22

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)

Answer 22

Goodpasture Syndrome

Question 23

Who gets Goodpasture syndrome?

Answer 23

males > females

teens to 20s

Question 24

Diagnostic linear Ig and complement deposits in basement membrane

Answer 24

Goodpasture syndrome

Question 25

Clinical features of Goodpasture syndrome?

Answer 25

initial presentation with hemoptysis
x-ray = focal pulmonary consolidations
renal manifestations follow

Question 26

How to treat Goodpasure syndrome?

Answer 26

Renal replacement therapy (dialysis) for severe AKI
Plasmapheresis to remove circulating anti-BM antibodies
immunosuppression

Question 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

Answer 27

Idiopathic pulmonary hemosiderosis

Question 28

Necrotizing vasculitis
Acute necrotizing granulomas
Necrotizing or granulomatous vasculitis
Renal disease with focal necrotizing, GN
Presents with hemoptysis
Often diagnosed by transbronchial biopsy

Answer 28

Wegener granulomatosis

Question 29

Engorged alveolar capillaries

Intra-alveolar pink precipitate

Answer 29

cardiogenic pulmonary edema

Question 30

What type of cells are seen in cardiogenic pulmonary edema?

Answer 30

“heart failure cells”

hemosiderin-laden macrophages from alveolar microhemorrhages

Question 31

Clinical signs of cardiogenic pulmonary edema

Answer 31

SOB
dyspnea on exertion
orthopnea
crackles on auscultation

Question 32

Where does fluid usu accumulate first in pulmonary edema?

Answer 32

in lower lobes

Question 33

What is one cause of noncardiogenic pulmonary edema?

Answer 33

acute lung injury/ARDS

Question 34

What will you see histologically with noncardiogenic pulmonary edema?

Answer 34

diffuse alveolar damage

Question 35

List four things that can cause acute lung injury, or ARDS?

Answer 35

sepsis
diffuse pulmonary infections
gastric aspiration
mechanical trauma, including head injuries

Question 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

Answer 36

diffuse alveolar damage

Question 37

What is the pathogenesis behind ARDS?

Answer 37

systemic/local insult
endothelial/epithelial damage: increased vascular permeability, alveolar flooding, reduced diffusion capacity, microthrombi
imbalance b/w pro and anti-inflammatory mediators

Question 38

Clinical features of ARDS?

Answer 38

rapid onset
profound dyspnea
tachypnea
severe cyanosis
respiratory failure
diffuse bilateral pulmonary infiltrates
very sick