KEY wk 6 lec 2 Flashcards
pulmonary pressures
very low for gas exchange
25/8mmHg
pulmonary hypertension
mean pulmonary artery >20 mmHg (use to be >25)
the mean pulmonary arterial pressure (mPAP) if healthy is 15mmHg
PAWP (pulmonary artery wedge pressure) <15 mmHg
changes in artery walls in pulmonary arterial hypertesnion
mild: thicken media
severe: intimal fibrosis and muscle thickening
grade 1, 2,3 are reversible, 4 is not
telangiectatic dilations (thin wall)
plexiform lesions
lumen obliteration and loose collagen
organized thrombus
6 types of pulmonary hypertension from sixth world symposium
- PAH
- Left heart disease
- Chronic lung
disease or sleep- disordered breathing - CTEPH
- Miscellaneous
primary pulmonary hypertension
pre capillary disorder; increase pulmonary arterial tone
idiopathic, hereditary, drugs or toxins that cause endothelial dysfunction, vascular remodel, resistnace ..
idiopathic pulmonary artery hypertesnion can lead to
more common in
leads to an increase in
right heart failure and early mortality
women
total pulmonary vascular resistnace (from changes in pulmonary arterial compliance)
To preserve cardiac output (CO) in the face of elevated right ventricular afterload, right ventricular work must increase.
sx of pulmonary artery hypertesnion
dyspnea
end stage: cardiac output declines and increase mPAP
extrapulnoanary vascular manifestations ie renal failure
systemic: increase aldosterone, angiotensin, estrogen, right ventricle fibrosis and dilation, muscle atrophy
cor pulmonale (death)
tx for pulmonary arterial hypertesnion
5 year survival via prostacyclin analogs, endothelin receptor antagonists and phosphodiesterase-5 inhibitors
usually do heart-lung transplant
pulmonary hypertension and left heart disease cause PWAP
> 15mmHg
pulmonary edema and causes
fluid accumulate in interstitial and alveolar spaces of lung
-increase capillary hydrostatic pressure (i.e. MI, heart fail)
-increased capillary permeability (i.e. sepsis, radiation)
-decreased colloid osmotic pressure
interstitial into alveolar pulmonary edema
pressure increases so much in interstitial space that it will fluid spill into alveoli
increase permeability and cause protein and red cells in alveolar fluid
interstitial vs alveolar edema
interstitial has little effect on pulmonary function
alveolar - cause alveoli to collapse and prevent ventilation; hypoxemia
sx of pulmonary edema
dyspnea and orthopnea (worse laying down)
suffocate or drown
cough with sputum
palpitations
anxiety
cold clammy
wheeze or gasp for breath
drugs for pulmonary edema
Diuretics
Inotrops
Antihypertensives
Morphine (acutely)
pulmonary hypertesnion associated with lung disease examples
COPD, interstitial lung disease, cystic fibrosis, sleep related respiratory disorders
pulmonary hypertesnion associated with lung disease changes in mPAP
pulmonary hypertesnion modest i.e. mPAP >20mmHG
venous thromboembolisms is 2 things
deep vein thrombosis (DVT) and pulmonary embolism (PE)
virchows triad for venous thrombi
- stasis of blood
- hypercoagulability
- intimal/endothelial injury
genetic mutations and aqduired causing hypercoagulability
factor V leidin (resistnace to activate protein C which should inactivate clotting factors)
prothrombin gene mutation (deficient in antithrombin, protein C and S which should stop coagulation)
acquired: antiphospholipid antibody syndrome
inflammation and triggers of DVT and PE
cancer, obese, smoking, OCP, pregnancy
psoriasis, T2DM, sepsis, pneumonia, lp(a)…
pulmonary embolism
thrombi from large veins travel to lungs and occlude pulmonary circulation
2 types: venous thrombi and nonthrombotic emboli (fat, air, amniotic fluid)
venous thromboembolism vs atherothrombosis
MI, PE and stroke overlap with likelihood and hyper coagulable, and inflam
atherothromsiss is atheroscletoritc plaque ruptures and cause thrombi (blood clot) and ischemia into stroke or heart attack
pulmonary embolism pathophysiology
right side of heart pumps deoxygenate blood to lungs
increase RV pressure and wall tension which causes dysfunction and ischmie
then decrease coronary perfusion and systemic pressure
can lead to decrease left ventricle preload and cardiac output
2 types of acute pulmonary embolism
- massive (high risk)
–> systemic arterial hypotension and thrombosis affects 1/2 of pulmonary vasculature (dyspnea, syncope, hypertension, cyanosis, cardiogenic shock - submassive (intermediate risk)
–> RV dysfunction but normal systemic arterial pressure - low risk (75% of cases)
massive vs submissive pulmonary embolism
massive: hypotension, RV fail –> cardiac shock
submassive: no hypotension, RV dilated but not failed
small vs medium vs massive emboli
small: maybe pulmonary hypertesnion
medium: dyspnea, pleural friction rub, near normal chest radiograph
massive: hemodynamic collapse (shock, pallor, cardiac arrest), hypotension
chronic thromboembolic disease (CTEPH) causes
pulmonary hypertension
shows fibrosis, artery stricture, webbing of arteries, dearborization
cor pulmonale definition
causes
right ventricle fails bc of high pulmonary artery pressures
-pulmonary emboli
-pulmonary vascular disease
-parenchymal disease (i.e. COPD)
changes in right ventricle in car pulmonale
hypertrophy, thicker wall, dilated, remodelling (lose septum curvature)
miscellaneous causes of pulmonary hypertesnion
sarcoidosis (mutlisystem inflam with granulomas but can be in lungs)
sickle cell disease (hemolysis and hypoxemia and high CO)
schistosomiasis (parasite cause granulomas and portal hypertesnion from hepatosplenic disease)
pre capillary vs post capillary
pre: increase pressure in pulmonary arteries before blood reaches lungs (i.e. PAH, CTEPH)
post capillary: increase pressure in pulmonary veins or left side of heart aging pressures in arteries that are downstream from it (I.e.left sided heart failure, )
capillary is in the pulmonary (i.e. sarcoidosis, asthma)
vascultitis
inflamed and damaged blood vessels
ischemia of tissues
granuloma, eosinophil, neutrophil, lymphoplasmacytic patterns
single organ or many
vasculitis classified by vessel size
large: aorta
medium: visceral arteries, i.e. renal, hepatic
small vessels: capillaries, venules, arterioles
lung involvement is seen in which type of vasculitis most
primary, idiopathic, small vessel
or antineutrophil cytoplasmic antibody associated vasculitides:
-wegeners granulomatosis
-microscopic polyangitis
-churg-strauss syndrome
Allergic Granulomatosis and Angiitis (Churg-Strauss Syndrome)
small and medium arteries are necrotizing –> thrombosis and aneurysm
granulomas and eosinophils
asthma
treat with corticosteroids
Granulomatosis with Polyangiitis (formerly Wegener Granulomatosis)
necrotizing vasculitis
granulomas
ANCA+
persistent bilateral pneumonitis, with nodular infiltrates that undergo cavitation
involves many systems (i.e. lungs, kidneys, otitis media, sinusitis, eyes vision loss and conjunctivitis, skin, fever, weight loss, neuropathy…)
sarcoidosis
mutlisystemic; non-necrotizing non-caseating granulomatous inflammation (lungs and intrathoracic lymph common)
genes and envo
infectious or non infectious triggers
who’s sarcoidosis most common in and associated with
40-55yoa
black
twins
genes
insecticides, cigarrets, microbes (mycobacterium)
pathology of sarcoidosis
noncaseating granulomatous infalmmation
T cells and monocytes
lung: multinucleated giant cells, and mononuclear cell inflammation
lymph: epithelioid granulomas
immunopathogeneiss of sarcoidosis
APC and Th cell complex release cytokines that form granulomas which can resolve or cause fibrosis
cytokines and circulating mediators go to organ and damage them via granulomas
symptoms of fever, weight loss, fatigue, pain…
stages of sarcoidosis
stage 0= no signs on x ray
stage 1= granuloma in lymph
stage 2- granuloma in lymph and lung
stage 3= granuloma in lung
stage 4= pulmonary fibrosis on x ray
lofgrens syndrome stage 1 of sarcoidosis
erythema nodosum (red skin bumps)
hilar adenopathy (enlarged lymphs at hilum of lungs)
periarticular arthritis
Stage 2 of sarcoidosis – Pulmonary Sarcoidosis Granulomas in Lymph Nodes & Lungs
stand away hilar nodes
paratracheal adenopathy (enlarged lymphs near trachea)
reticulonodular infiltrates
Stage 3 & 4 – Pulmonary Sarcoidosis Granulomas - Fibrosis
fibrocystic sarcoidosis
lung scarring, bulbous and cystic changes, hilar retraction
parenchymal infiltrates
pulmonary function test in sarcoidosis
restrictive impairment; reduce FVC and FEV1
reduced diffusing capacity
obstructive impairment (advanced fibrocystic disease)
resting hypoxemia and exercise O2 desaturation
pulmonary hypertension
clinical manifestations of sarcoidosis
pulmonary: reduced diffusing capacity, restrictive, obstructive, fibrocystic
constitutional: fever, sweat, fatigue
Upper respiratory tract: hoarseness, laryngeal obstruction, sinusitis
skin: nodules and plaques, alopecia, lesion on nose, eyes, cheek, maculopapular lesions on trunk
ocular
abdominal: spleen, rib and spine increased activity on CT and PET
cardiac: arrhythmia, death
lupus pernio
extra pulmonary sarcoidosis
causing lesions on nose, eyes, cheek
cardiac sarcoidosis
i.e. AV block, sudden cardiac death, congestive heart failure, ventricular tachycardia
Progressive Systemic Sclerosis (Scleroderma) effects the lungs how
pulmonary fibrosis, intestinal lung disease via connective tissue autoimmune, pulmonary hypertension
systemic Lupus Erythematosus effects the lungs how
pleuritis, pulmonary infiltrates, interstitial inflammation leading to fibrosis, shrinking lung syndrome, intra-alveolar hemorrhage, pulmonary arterial hypertension
how rheumatoid arthritis affects the lungs
pleuritis
fibrosis- interstitial lung disease
respiratory failure
pulmonary hypertesnion
occupational lung diseases
inorganic vs organic dusts
inorganic
- asbestos
-silica
-coal dust
-metals
–> i.e. fibrosis, cancer,
organic:
-cotton
-grain
-agriculture
–> ie.. asthma, bronchitis, COPD
asbestos-related lung disease
complications
primary exposure: miners and millers
secondary exposure: manufacturing plants
complications;
-nonmalignant pleural
-asbestosis
-malignant mesothelioma
-lung cancer
asbestosis
initial macrophage alveolitis
if fibers of asbestos arent cleared then cause fibrosis
interstitial pneumonitis and fibrosis
fibrosis on walls of respiratory bronchioles
septa thicken
clinical prevention of asbestosis
parenchymal fibrosis
dyspnea, bibasilar rales
restrictive ventilatory impairment
silicosis
alveolitis
ground glass appearance
calcify hilar nodes
process to cause obstructive and restrictive ventilatory impairment
progressive massive silicosis
Obstructive & restrictive ventilatory impairment
Respiratory failure
rheumatoid arthritis and scleroderma
lung infections: mycobacterium tuberculosis
mesothelioma (cancer)
coal workers pneumoconiosis
simple: chronic bronchitis and COPD
complicated: Caplan syndrome of coal/silica and rheumatoid arthritis
pathology in coal worker pneumoconiosis
-coal macule
-collect coal dust in macrophages
-centriacinar emphysema
sx in coal worker pneumoconiosis
cough and sputum, dyspnea
obstructive, restrictive
emphysema, fibrosis
reduce gas exhange
hypoxia, pulmonary arterial hypertesnion
beryllium disease (work exposure)
acute pneumonitis
chronic granulomatous disease (become sensitized and then get a positive BeLPT)
sensitization phase (bc hypersensitivity) –> chronic
