Pulmonary Vascular Disease

Question 1

genetics of IPAH (primary pulmonary HTN)

Answer 1

long arm chromosome 2 2q31-32

BMPR2 gene stop codon exon mutations

AD with low penetrance

Question 1

system pathologies, wegeners

Answer 1

systemic vasculitis > granulomatous inflammation

upper respiratory (then lower)

Kidney

Question 2

symptoms Wegener’s

Answer 2

nasal sinus otitis

cough, hemoptysis

constitutional (joints, fever)

Question 3

best non-invasive diagnositc for PH

Answer 3

echocardiogram

Question 3

group I pulmonary artery htn =

Answer 3

precapilary

(idiopathic_

Question 4

ANCA associated vasculitis =

Answer 4

wegner’s

Question 5

associated with APAH

Answer 5

Collagen vascular disease (Scleroderma, SLE, RA)

congenital heart disease

portal HTN

HIV

Drugs

Question 6

samples from ____ show more apparent hemosiderian macrophages, blood (if present)

Answer 6

deep in lung

Question 6

demographics wegener’s

Answer 6

50yo male > female

Question 7

antibasement membrane antibody disease =

Answer 7

goodpastures

Question 9

poor prognosis in PH at (pressure levels)

Answer 9

RA pressure >10mmHg

CI < 2.2L/min²

Question 10

pathways implicated in PAH pathogensis

Answer 10

increased endothelian

decreased NO pathway

decreased Prostacyclin pathway

Question 12

group IV pul HTN =

Answer 12

chronic thrombotic, embolic diseases

(obstruction of proximal, distal arteries)

Question 13

classic triad alveolar, capillary hemorrhage syndromes

Answer 13

hemoptysis

pulmonary infiltrates

enemia

Question 15

Venous thromboembolism risk factors

Answer 15

virchows triad: stasis, injury, hypercoaguabilty

Thrombophilias (Prot C, S, ATIII, Factor V Leiden, prothrombin mutation, MTHFR, Factor VIII

**Medical risks: **hip/knee surgery, CHF, obesity, Malignancy, acquired hypercoag (CA)

Question 16

symptoms pulmonary embolism

Answer 16

dypsnea

dizziness, syncope

pleuritic chest pain

palpitations

tachycardia

hemotyisis

Question 16

group I PAH definition (defining measures)

Answer 16

mean pulmonary arterial pressure (mPAP) >25mmHg at rest with normal wedge rpressure

Question 17

regions of pulmonary involvement, wegener’s granulomatosis

Answer 17

Capillaritis, Alveolar Hemorrhage

nodules

cavitary lesions

large airways

interstitial lung disease

Question 18

diagnosis alveolar capillary hemorrhage syndromes

Answer 18

sputum, tacheal aspirate

urine (other organ inmpact)

serial Hgb - -2.0g in 24 hours

serial CXR

**serologies **(ABMab, ANCA, ANA)

Broncheolar lavage/open lung biopsy

Question 20

pulmonary vasculitis pulmonary presentation

Answer 20

pulmonary+alveolar hemorrhage

lung infiltrates (nodular, cavitary lesions, associated ILD, bilateral diffuse)

pul HTN

Question 21

life threatening complications in Aveolar capillary hemorrhage syndromes

Answer 21

resp failure

acute renal failure

severe anemia

Question 22

most imporant factor in Pul HTN prognosis

worst class?

Answer 22

level of physical activity (functionall assessment)

class IVis worst

Question 23

hissto wegener’s

Answer 23

preivascualr necrotizing granuloamtous inflammation

lung Necrosis, Cavitation, Hemorrhage

Question 24

incompressible vein implies

Answer 24

clot proximal to area

Question 25

morphology Group I pulmonary arterial Htn

Answer 25

large pul artery thickening

medial wall smooth muscle hypertrophy

plexiform lesion in small vessels (specific to group 1)

Question 26

most common cause of PH

Answer 26

left heart disease

Question 27

“keyhole” to APAH

Answer 27

BMPR2 mutations