Pulmonary vascular disease

Question 1

Under normal conditions, interstitial spaces of the lung is kept dry by

Answer 1

Pulmonary lymphatics located within the axial and peripheral interstitium of the lung

Question 2

Because there are no lymphatic structures immediately within the alveolar walls (parenchymal interstitium), filtered interstitial fluid is drawn to lymphatics by a

Answer 2

Pressure gradient from alveolar interstitium to the axial and peripheral interstitium

Question 3

When the rate of fluid accumulation in the interstitium exceeds the lymphatic drainage capabilities of the lung, fluid accumulates first within the

Answer 3

Interstitial spaces

Question 4

Most common mechanism of pulmonary edema

Answer 4

Change in the normal starling forces that govern fluid movement in the lung

Question 5

Imbalance of starling forces in pulmonary edema is most commonly the result of

Answer 5

Increase capillary hydrostatic pressure, and less commonly diminished plasma oncotic or interstitial hydrostatic pressure

Question 6

Other mechanisms in pulmonary edema aside from imbalance in starling forces

Answer 6

• obstruction or absence of the normal pulmonary lymphatics, which leads to the excess accumulation of interstitial fluid.
• disorders can injure the epithelium of the capillaries and alveoli, causing an increase in capillary permeability that allows protein-rich fluid to escape from the capillaries into the pulmonary interstitium

Question 7

Thickening of axial interstitium results in what imaging findings

Answer 7

Loss of definition of intrapulmonary shadows and thickening of peribronchovascular interstitium causing peribronchial cuffing and tram tracking

Question 8

Involvement of peripheral and subpleural interstitial structures in pulmonary edema produces

Answer 8

Kerley lines and subpleural edema

Question 9

Represents thickening of central connective tissue septa and peripheral interlobular septa, respectively

Answer 9

Kerley A and B lines

Kerley C lines represent a network of thickened interlobular septa

Question 10

It is the accumulation of fluid within the innermost (interstitial) layer of the visceral pleura

Answer 10

subpleural edema

Question 11

subpleural edema are is best seen in ____ and appears as smooth thickening of the interlobular fissures

Answer 11

lateral radiographs

Question 12

Develops when fluid in the interstitial spaces extends into the alveoli

Answer 12

Airspace pulmonary edema

Question 13

Most common form of pulmonary edema

Answer 13

Hydrostatic pulmonary edema

Question 14

Hydrostatic pulmonary edema is usually caused by

Answer 14

elevation in pulmonary venous pressure (pulmonary venous hypertension)

Question 15

Causes of pulmonary venous hypertension may be divided into four major categories

Answer 15

• obstruction to left ventricular inflow,
• left ventricular systolic dysfunction (LV failure),
• mitral valve regurgitation and
• systemic or pulmonary volume overload

Question 16

classic cause of obstruction to left ventricular inflow is

Answer 16

mitral stenosis

Question 17

more common causes of LV inflow obstruction

Answer 17

poor left ventricular compliance (diastolic dysfunction), such as caused by hypertrophy or chronic ischemic subendocardial fibrosis

Question 18

3 Common causes of LV failure include

Answer 18

• ischemic heart disease,
• aortic valve stenosis and regurgitation, and
• nonischemic cardiomyopathy

Question 19

Normal pcwp

Answer 19

8-12 mmHg

Question 20

Pcwp level that leads to findings of interstitial pulmonary edema such as loss of vascular definition, peribronchial cuffing and Kerley lines

Answer 20

19-25 mmHg

• Mild elevation of PCWP (12 to 18 mm Hg) produces constriction of lower lobe vessels and enlargement of upper lobe vessels.
• Progressive elevation of PCWP (19 to 25 mm Hg) leads to the findings of interstitial pulmonary edema:
  • loss of vascular definition,
  • peribronchial cuffing, and
  • Kerley lines.

Question 21

Pcwp level that produces alveolar filling with radiographic findings of bilateral airspace opacities in the perihilar and lower lung zones

Answer 21

More than 25 mmHg

Question 22

classic radiographic findings of Pulmonary Venous Hypertension

Answer 22

enlargement of pulmonary veins and redistribution of pulmonary blood flow to the nondependent lung zones

Question 23

True or false: with PVH in the upright patient with normal lung parenchyma, the upper zone vessels are frequently as large as or larger in diameter than the lower zone vessels

Answer 23

true

Question 24

alveolar pulmonary edema localized to the right upper lung may be seen in patients with

Answer 24

severe mitral regurgitation

Question 25

When respiratory failure develops as a result of this condition, and is associated with increased lung stiffness (noncompliance) it is termed

Answer 25

acute lung injury or when severe, ARDS

Question 26

6 most common causes of increased permeability edema

Answer 26

``` shock, severe trauma, burns, sepsis, narcotic overdose and pancreatitis ```

Question 27

key factors in the development of capillary endothelial damage

Answer 27

recruitment and activation of neutrophils in the lung, with release of enzymes and oxygen radicals

Question 28

Stage of ARDS: happens within 12-24 hours following initial insult, damage to capillary endothelium produces engorged capillaries and proteinaceous interstitial edema

Answer 28

Stage 1: exudative

Question 29

Stage of ARDS: within the first week, injury to type 1 pneumocytes leads to flooding of alveoli with edema fluid and proteinaceous and cellular debris, which form hyaline membranes lining the distal airways and alveoli

Answer 29

Stage 2: proliferative

Question 30

Stage of ARDS: occurring 10 to 14 days following the initial insult, type 2 pneumocytes proliferate in an attempt to reline the denuded alveolar surfaces and fibroblastic tissue proliferates within the interspaces

Answer 30

Stage 3: fibrotic

Question 31

Radiographic pattern of ARDS

Answer 31

patchy peripheral airspace opacities by 12-24 hours, with minimal or absent interlobular septal thickening, coalesce over the next days to produce confluent bilateral airspace opacities with air bronchograms, after a week becomes coarse reticulonodular, if unchanged, honeycombing

Question 32

in pulmonary edema associated with chronic cardiac failure, the vascular pedicle, which represents the mediastinal width at the level of SVC and left subclavian artery measures

Answer 32

>53 mm on PA radiograph

Question 33

90% of all primary tracheal tumors in adults are malignant or benign?

Answer 33

Malignant

Question 34

Most common histologic type of primary tracheal malignancy

Answer 34

Squamous cell carcinoma

Question 35

Majority of tracheal malignancy arise in what area

Answer 35

Distal trachea, within 3-4 cm of tracheal carina

Question 36

Second next common site in tracheal neoplasms

Answer 36

Cervical trachea

Question 37

Malignant neoplasm that arises from the tracheal salivary glands and accounts for 40% of primary tracheal malignancies. Tends to involve the posterolateral wall of the distal 2/3 of trachea or main or lobar bronchi

Answer 37

Adenoid cystic carcinoma

Question 38

Arises from tracheal cartilage and is identified by the presence of calcified chondroid matrix within the tumor

Answer 38

Chondrosarcoma

Question 39

Most common type of thyroid malignancy to invade the trachea

Answer 39

Papillary and follicular

Question 40

Extrathoracic primary tumors that are most often associated with hematogeneous endotracheal metastases are carcinomas of

Answer 40

Breast, kidney and colon and melanoma

Question 41

capillary permeability edema can sometimes be distinguished from hydrostatic edema by the following:

Answer 41

a non-dependent or peripheral distribution of edema, absence of other signs of hydrostatic edema such as interlobular septal thickening and subpleural edema, and a lack of short term change

Question 42

true or false: in neurogenic pulmonary edema, both hydrostatic and increased permeability mechanisms are involved

Answer 42

true

Question 43

high altitude pulmonary edema happens in individuals who had rapid ascent of altitudes above

Answer 43

3500 m

Question 44

Pathophysiology of high altitude pulmonary edema

Answer 44

edema typically develops within 48-72 hours of ascent and appears to reflect a varied individual response to hypoxemia, in which scattered areas of pulmonary arterial spasm result in transient pulmonary arterial hypertension---> causes damage to capillary endothelium and increase permeability edema, typically with a patchy distribution

Question 45

more susceptible to reexpansion pulmonary edema

Answer 45

rapid evacuation of large pneumothorax or PE present for more than 48 hours and young age

Question 46

Pulmonary edema may also happen after immediate treatment of acute upper airway obstruction due to

Answer 46

creation of markedly negative intrathoracic pressure by attempts to inspire against an extrathoracic airway obstruction, producing decreased interstitial hydrostatic pressure, thereby promoting transudation of fluid into lungs

Question 47

severe and often fatal form of pulmonary edema tha may develop in a pregnant woman when amniotic fluid gains access to the systemic circulation during labor

Answer 47

amniotic fluid embolism

Question 48

fetal distress and demise may happen in amniotic fluid embolism because of ___ which play a key role in the pathogenesis of this disorder

Answer 48

mucin within fetal meconium

Question 49

contributes to vascular collapse leasding to sudden PAH, cor pulmonale with decreased CO and pulmonary edema in amniotic fluid embolism

Answer 49

anaphylactoid reaction and DIC

Question 50

There may be enlargement of central pulmonary arteries and right heart as manifestation of

Answer 50

cor pulmonale

Question 51

common complication occurring 24 to 72 hours after the fracture of a long bone such as the femur

Answer 51

fat embolism

Question 52

mechanism of pulmonary edema in fat embolism

Answer 52

fat is hydrolyzed to its component fatty acids causing increased pulmonary capillary permeability and hemorrhagic pulmonary edema

Question 53

systemic findings of fat embolism

Answer 53

petechial rash, CNS depression and pulmonary changes

Question 54

6 Autoimmune diseases associated with pulmonary hemorrhage

Answer 54

``` Goodpasture syndrome, idiopathic pulmonary hemorrhage, granulomatosis with polyangiitis, sytemic lupus erythematosus, RA and polyarteritis nodosa ```

Question 55

autoimmune disease characterized by damage to the alveolar and renal glomerular basement membranes by a cytotoxic antibody. the antibody is directed primarily against renal glomerular basement membrane and cross reacts with alveolar basement membrane to produce renal injury and pulmonary hemorrhage characteristics of this disorder

Answer 55

Goodpasture syndrome

Question 56

diagnosis of Goodpasture disease

Answer 56

immunofluoresent studies of renal or lung tissue

Question 57

development of pulmonary hemorrhage in vasculitides such as granulomatosis with polyangitis, SLE, RA and polyarteritis nodosa are due to

Answer 57

small vessel pulmonary arteritis and capillaritis which results in spontaneous hemorrhage

Question 58

main laboratory test in patients with suspected pulmonary embolism

Answer 58

plasma D-dimer level

Question 59

It is a degradation product of fibrin and is a very sensitive indicator of the presence of venous thrombosis

Answer 59

D-dimer

Question 60

First examination obtained in all patients with suspected PE

Answer 60

chest radiograph

Question 61

most common radiographic findings in pulmonary embolism without infarction are

Answer 61

peripheral airspace opacities and linear atelectasis

Question 62

Known as the westermark sign which is extremely rare finding in pulmo embolism

Answer 62

localized peripheral oligemia with or without distended proximal vessels

Question 63

known as Hampton hump in infarction related to pulmo embolism

Answer 63

presence of pleural effusion and the development of a pleura-based wedge-shaped opacity which lacks air bronchogram

Question 64

Hampton hump in pulmo embolism is usually seen in what area

Answer 64

posterior or lateral costophrenic sulcus of the lung

Question 65

in embolism without infarction, airspace opacities should resolve completely within how many days

Answer 65

7-10 days

Question 66

first imaging study to be done in pregnant women with a normal chest radiograph in the diagnosis of pulmo embolism

Answer 66

Ventilation/perfusion lung scintigraphy using IV macroaggregated albumin radiolabeled with technetium (Tc-99m)

Question 67

Recommended study for detection of pulmonary embolism

Answer 67

CT angiography of pulmonary arteries

Question 68

CT pulmo angiography is better done in what phase of respiration

Answer 68

expiratory

Question 69

chronic emboli should be suggested when

Answer 69

filling defect is adherent to the vessel wall rather that in the center of lumen or when a web is present

Question 70

technique selectively used whena definitive diagnosis of pulmo embolism or DVT cannot be achieved by less invasive means. It requires right heart and pulmonary artery catheterization

Answer 70

digital subtraction angiography

Question 71

3 secondary signs of pulmo embolism in pulmonary angiography include

Answer 71

prolonged arterial phase, diminished peripheral perfusion and delay in the venous phase

Question 72

causes of nonthrombotic pulmonary embolism

Answer 72

air embolism, fat embolism, methylmethacrylate embolization complicating vertebroplasty, radioactive seed implant embolization from prostate brachytherapy

Question 73

pulmonary tumor emboli usually comes from what 4 malignancies

Answer 73

renal cell carcinoma, breast cancer, HCC and GI malignancy

Question 74

pulmonary arterial hypertension is defined as a systolic pressure in the pulmonary artery exceeding ___ mmHg either measured directly by right heart catheterization or as estimated by echocardiography

Answer 74

30

Question 75

typical radiographic findings of PAH

Answer 75

enlarged main and hilar pulmonary arteries that taper rapidly toward the lung periphery

Question 76

useful measurement for enlargement of central pulmonary artery, usually indicating PAH in the absence of a left-to-right shunt is a transverse diameter of proximal interlobar artery on PA radgiograph that exceeds

Answer 76

16 mm

Question 77

in patients younger than 50 years, in the diagnosis of PAH, a ratio of diameter of the MPA( measured at the level of the main Right PA) to the transverse diameter of ascending aorta at the same level greater than 1 strongly correlated with a mean PA pressure greater than

Answer 77

20 mmHg

Question 78

In patients older than 50 years, a maximum transverse measurement of MRA greater than ___ mm correlates better with PAH

Answer 78

30

Question 79

Flattening or bowing of the interventricular septum toward the LV indicates

Answer 79

RV hypertension

Question 80

Signs of long standing PAH

Answer 80

RV hypertrophy, RV dilatation, and right heart failure (cor pulmonale)

Question 81

disease among young women in whom medial hypertrophy and intimal fibrosis obliterate the muscular arteries

Answer 81

plexogenic pulmonary arteriopathy

Question 82

a condition related to PVOD which is characterized by the proliferation of capillaries throughout the pulmonary interstitium, resulting in venular obstruction

Answer 82

pulmonary capillary hemangiomatosis

Question 83

combination of pulmonary edema with a normal sized heart, absent findings for PVH, normal PCWP and insiduous onset of dyspnea should suggest this diagnosis rather than left heart failure, mitral valve disease or large vessel pulmonary venous occlusion

Answer 83

pulmonary capillary hemangiomatosis/PVOD

Question 84

radiographic findings in PCH/PVOD

Answer 84

interlobular septal thickening, centrilobular nodular ground-glass opacities, and pleural effusions