Pul Vascular Disease Flashcards

1
Q

genetics of IPAH (primary pulmonary HTN)

A

long arm chromosome 2 2q31-32

BMPR2 gene stop codon exon mutations

AD with low penetrance

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2
Q

system pathologies, wegeners

A

systemic vasculitis > granulomatous inflammation

upper respiratory (then lower)

Kidney

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3
Q

symptoms Wegener’s

A

nasal sinus otitis

cough, hemoptysis

constitutional (joints, fever)

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4
Q

group I pulmonary artery htn =

A

precapilary

(idiopathic_

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5
Q

best non-invasive diagnositc for PH

A

echocardiogram

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6
Q

ANCA associated vasculitis =

A

wegner’s

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7
Q

associated with APAH

A

Collagen vascular disease (Scleroderma, SLE, RA)

congenital heart disease

portal HTN

HIV

Drugs

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8
Q

demographics wegener’s

A

50yo male > female

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9
Q

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

A

deep in lung

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10
Q

antibasement membrane antibody disease =

A

goodpastures

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11
Q

poor prognosis in PH at (pressure levels)

A

RA pressure >10mmHg

CI < 2.2L/min2

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12
Q

pathways implicated in PAH pathogensis

A

increased endothelian

decreased NO pathway

decreased Prostacyclin pathway

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13
Q

group IV pul HTN =

A

chronic thrombotic, embolic diseases

(obstruction of proximal, distal arteries)

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14
Q

classic triad alveolar, capillary hemorrhage syndromes

A

hemoptysis

pulmonary infiltrates

enemia

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15
Q

Venous thromboembolism risk factors

A

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)

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16
Q

group I PAH definition (defining measures)

A

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

17
Q

symptoms pulmonary embolism

A

dypsnea

dizziness, syncope

pleuritic chest pain

palpitations

tachycardia

hemotyisis

18
Q

regions of pulmonary involvement, wegener’s granulomatosis

A

Capillaritis, Alveolar Hemorrhage

nodules

cavitary lesions

large airways

interstitial lung disease

19
Q

diagnosis alveolar capillary hemorrhage syndromes

A

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

20
Q

pulmonary vasculitis pulmonary presentation

A

pulmonary+alveolar hemorrhage

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

pul HTN

21
Q

life threatening complications in Aveolar capillary hemorrhage syndromes

A

resp failure

acute renal failure

severe anemia

22
Q

most imporant factor in Pul HTN prognosis

worst class?

A

level of physical activity (functionall assessment)

class IVis worst

23
Q

hissto wegener’s

A

preivascualr necrotizing granuloamtous inflammation

lung Necrosis, Cavitation, Hemorrhage

24
Q

incompressible vein implies

A

clot proximal to area

25
Q

morphology Group I pulmonary arterial Htn

A

large pul artery thickening

medial wall smooth muscle hypertrophy

plexiform lesion in small vessels (specific to group 1)

26
Q

most common cause of PH

A

left heart disease

27
Q

“keyhole” to APAH

A

BMPR2 mutations