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Path Pulmonary Vascular
KYCOM Block 15
51
Pathology
Graduate
04/03/2014

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Cards

Term
risk factors of pulmonary thrombiemboli 7
Definition
prolonged bed rest
surgery (esp orthopedic of knee and hip)
severe trauma (esp burn, multiple fractures)
congestive heart failure
oral contraceptives (esp high estrogen)
disseminated cancer
primary hypercoaguability
Term
what is the main determinant of which artery a thromboemboli will occlude in lung
Definition
cardiopulmonary stats of patient
size of emvoli
Term
where do most pulmonary thrombi come from
Definition
95% come from DVT in lower popliteal or larger veins above knee
Term
complications of a blocked pulmonary artery 7
Definition
increased pressure
neurogenic induced vasospacm
ischemia downsteam parenchyma
decreased CO, right side heart failure, sudden death
hypoxemia
Term
explain how pulmonary thrombi causes hypoxemia
Definition
perfusion of lung zones becomes atlectatic (alveoli collapse due to ischemia reducing surfactant production and causing pain so chest wall dosent move as much)

hypoxic areas clamp off and shunt blood to areas of the lung normally hypoventilated

this decreases CO and widens difference in arterial venous O2 saturation (can induce R to L shunt in PDA)
Term
why isnt ischemic necrosis a complication of a blocked pulmonary artery
Definition
bronchial artery provides blood flow back up
Term
what are the 4 different types of pulmonary emboli and where do they land
Definition
saddle: main pulmonary artery or bifurcation
small emboli: medium and small pulmonary arteries
medium emboli: causes pulmonary infarct of medium arteries
recurrent multiple: around lung causing HTN
Term
what are the consequences of a pulmonary infarct that is saddle 3, small 2, medium 1, recurrent multiple 4
Definition
saddle: death from hypoxemia, acute cor pulmonale

small: alveolar hemorrhage due to ischemic endothelial damage, fibrinolutic activity breaks down (clinically silent), parenchyma spared due to bronchial artery

medium: pulmonary infarct

recurrent multiple: pulmonary HTN, cor pulomonale, sclerosis, dyspneia
Term
describe the lesion in a pulmonary infarct: shape, early and late appearance, why
Definition
wedge shape with base at pleural surface pointing to hilum and occlusion at apex

hemorrhagic: red/blue early, adjacent with pleura fibrous exudate

RBC lyse in 48h making it pale then red brown as hemosiderin builds up

over time fibrous replacement makes it gray-white then scars
Term
causes of nonthrombotic pulmonary emboli 5
Definition
air, fat, amniotic fluid, foreign body, marrow, talc
Term
how can you identify a talc pulmonary emboli 3
Definition
granulomatous response in interstitium of pulmonary arteries that causes fibrosis and HTN
Term
why do marrow pulmonary emboli occur 2
Definition
hematopoetic and fat cells after massive trauma or bone infarct secondary to sickle cell
Term
clinical signs of pulmonary infarct: multiple recirrent 4, medium 1, small 1, complications 5
Definition
small: usually clinical silent and removed by fibrinolysis

medium: circiulatory insufficiey

CV collapse, shock, right heart failure: chest pain, dyspnea (occurs when >60% pulmonary vasculature occluded)

recurrent multiple emboli: pulmonary HTN, chronic right heart strain, pulmonary vascularsclerosis, worsening dyspnea
Term
prophylaxis of pulmonary ischemia 5
Definition
anticoagulation
early ambulation for postop and postpartum patients
elastic stickings
intermittent calf compression
isometric leg exercises if bed ridden
Term
tx of pulmonary ischemia
Definition
small emboli recieve anticoagulation
large emboli recieve thrombotics
Term
prognosis of pulmonary iscemia
Definition
1/3 not diagnosed before death
normally resolve after initial acute event
predisposition factors make small emboli present as large
HISTORY OF PULMONARY EMBOLI INCREASES CHANGES OF ANOTHER BY 30%
Term
how is pulmonary HTN defined
Definition
increased pressure in the normally low resistance pulmonary circulation when pulmonary pressure reaches 1/4 or more the systemic levels (MAP>25 when normal is 10)
Term
what are the 3 categories of causes of secondary pulmonary HTN and some examples of diseases in each
Definition
hypoxemia: COPD, interstitial lung disease, athlerosclerosis of pulmonary trunk, fibrosis

recurrent pulmonary embolism

increased lung volume in pulmonary circut: mitral stenosis, congenital heart disease, eismengers
Term
how does hypoxemia lead to secondary pulmonary HTN
Definition
plexiform lesion: tufts of capillaries seen in long standing disease

destruction of parenchyma and reduction of alveolar capillaries increases pulmonary artery resistance and increases pressure
Term
how does recurrent pulmonary embolism cause secondary pulmonary HTN
Definition
reductionin cross sectional area of vessels increases resistance
long standing emboli thicken vascular wall causing HTN
Term
how does increaed volume in the pulmonary circut cause secondary pulmonary HTN
Definition
causes smooth muscle hypertrophy
Term
how does mitral stenosis cause pulmonary HTN
Definition
increases LA pressure and thus venous pressure
Term
signs of secondary pulmonary HTN
Definition
symptoms reflect underlying disease
seen at any age
respiratory insufficiency
right sided heart strain
Term
what ae the two causes of primary / idiopathic pulmonary HTN and their prevalence
Definition
familial 60%
sporatic 40%
Term
how does familial primary pulmonary HTN cause HTN
Definition
AD germline mutation morphogenic protein receptor type 2 (BMPR-2) binds to TGF-b causing abnormal vessel endothelial and lung smooth muscle proliferation

not all who have mutation have disease, modifier genes affect penetrance
Term
how does sporatic primary pulmonary HTN cause HTN
Definition
serotonin transporter gene (5HTT) on pulmonary smooth muscle cells have increased proliferation on serotinin exposure
Term
sigs of primary/idiopathic pulmonary HTN 9
Definition
young adult, usually women
fatigue, syncope (esp with exercise)
dyspnea on exertion, CP
2-5y later respiratory insufficiency, cyanosis, cor pulmonale
Term
explain the cellular changes that occur in pulmonary HTN 5
Definition
endothelial cell and smooth muscle dysfunction due to mechanical or biochemical injury

reduces vasodilators (NO, prostacyclin), increases vasoconstrictors (endothelial

produces GF and cytokines to induce margination and replication of vascular smooth muscle and ECM
Term
what changes occur in main elastic arteries in pulmonary HTN
Definition
atheromas similar to systemic athleroscleriosis
Term
what changes occur in the medium arteries in pulmonary HTN
Definition
proliferation of myointimal and smooth muscle cells thickens intima and media and narrows lumen
Term
what chages occur int he small arteries and arterioles in pulmonary HTN
Definition
thickening, medial hypertrophy, replication of elastic membranes
thickness may exceede diameter and obliderate lumen
Term
how can you distinguish primary vs secondary pulmonary HTN by morphology
Definition
idiopathic pulmonary HTN has plexiform lesions with proliferation in multiple lumina in small arteries where they branch into medium arteries
Term
3 tx for pulmonary HTN
Definition
vasodilators
antithrombolytics
prostacyclin infusion
Term
prognosis of pulmonary HTN
Definition
poor, will need lung transplant if due to chronic condition
Term
what is a diffuse alveolar hemmorhage syndrome
Definition
primary immune mediated disease with triad of hemoptysis, anemia, and diffuse pulmonary infiltrates
Term
what are 4 diffuse alveolar hemorrhage syndromes
Definition
goodpasture
idiopathic pulmonary hemosiderosis
pulmonary angiitis and granulomatosis (wegner granulomatosis)
limited wegner granulomatosis
Term
goodpastures: cause and effect
Definition
proliferative rapid progressive glomerulonephritis and hemorrhagic interstitial pneumonitis due to autoantibodies to noncollagenous domain a3 on collagen IV on BM
Term
what changes occur in lungs in goodpasture 7
Definition
diffuse alveolar hemorrhages: heavy, red brown consolidations, focal necrosis of alveolar walls, fibrous thickening of septa, hypertrophic type II pneumocytes, hemosiderin in macriphages and extracellular
Term
what changes occur in kidney in goodpasture
Definition
linear pattern of IgG deposition on renal biopsy (this can sometimes be seen in alveolar septa)
Term
how is goodpastures diagnosed
Definition
antibodies in serum of 90% of patients
Term
tx of goodpastures 3
Definition
plasmapharesis
immune supression
evuntal renal transplant
Term
idiopathic pulmonary hemosiderosis; epidemology
Definition
usually kids, but adults ahve better prognosis
Term
idiopathic pulmonary hemosiderosis; cause
Definition
probablly immune mediated (periodic inactivity and remission)
Term
morphology of idiopathic pulmonary hemosiderosis;
Definition
pulmonary histologically like goodpastures but no renal disease or anti BM antibody
diffuse alveolar hemorrhages
Term
tx of idiopathic pulmonary hemosiderosis 2, and prognosis
Definition
steroids
immune supression
improves survival by 2.5 y
Term
what is another name for wegner granulomatosis
Definition
pulmonary antgiitis and granulomatosis
Term
cause of wegener's granulomatosis
Definition
PR3-ANCAs present in 95%
Term
wegener's granulomatosis: morphology, location
Definition
necrotizing vasculitis (angiitis) and perenchymal necrotizing granulomatosis inflammation
Term
signs of wegener's granulomatosis 6
Definition
80% develop pulmonary upper respiratory signs: chronic sinusitis, epistaxis, nasal perforation, cough, hemoptysis, CP
Term
CXR in wegener's granulomatosis
Definition
multiple nodular densities (confluence of granulomas, some cavitated
Term
how is limited wegener's granulomatosis different than wegener's granulomatosis
Definition
same disease be restricted to the lung
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