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Definition
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Definition
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definition of pulmonary HTN |
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Definition
mean PA pressure >25mmHg at rest or >30mmHg at exercise |
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Term
pre-capillary causes of pulmonary HTN (4) |
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Definition
diseases affecting pulmonary aa., lung diseases, external compression, hypoventilation |
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post-capillary causes of pulmonary HTN (2) |
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Definition
left heart disease, veno-occlusive disease |
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PWCP - PA > 10mmHg -> pre or post capillary? |
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Definition
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PWCP - PA < 5mmHg -> pre or post capillary? |
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Definition
post--specifically heart disease |
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PWCP - PA = normal -> pre or post capillary? |
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Definition
post--specifically, veno-occlusive disease |
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Term
10% of pulmonary HTN cases; caused by defect in BMPR2 gene; dx? |
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Definition
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epidemiology of primary pulmonary HTN |
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Definition
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most common causes of pulmonary HTN (2) |
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Definition
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Term
how do kyphoscoliosis and sleep apnea cause pulmonary HTN? |
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Definition
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histological characteristics of idiopathic pulmonary HTN (4) |
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Definition
plexiform lesions, medial hypertrophy, intimal hyperplasia and fibrosis of medium-size aa. |
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when BMPR2 is not the cause, what pathogeneses contribute to pulmonary HTN? (3) |
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Definition
endothelial dysfunction, dysfunctional K+ channels, vasoactive amines |
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Term
which endothelin-1 receptor causes vasoconstriction? vasodilation? |
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Definition
ETA -> vasoconstriction; ETB -> vasodilation |
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Term
how does K+ channel dysfunction cause pulmonary HTN? |
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Definition
K+ channel dysfunction -> increase intracellular Ca++ -> increase vascular tone |
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Term
what factors increase pulmonary a. pressure? (3) |
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Definition
increased CO (volume/pressure overload), increased PVR (obstruction, vasoconstriction), and increase LA pressure (left heart disease) |
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how does left-to-right shunt contribute to pulmonary HTN? |
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Definition
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how does a large LA myxoma contribute to pulmonary hypertension? |
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Definition
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how does COPD, high altitude, and hypoventilation contribute to pulmonary HTN? |
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Definition
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diseases that obliterate pulmonary vasculature (4) |
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Definition
thrombo-embolism, vasculitis, emphysema, extrinsic compression |
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Definition
primary, familial, congenital, portal HTN, infection; always a candidate for advanced therapy |
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Definition
left heart disease; always work up for thrombo-embolic disease |
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Definition
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Definition
thrombo-embolic disease; dx via V/Q scan |
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pt presents with pulmonary HTN, vasculitis, skin lesions, low DLCO but normal PFTs; what group does this belong in? |
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Definition
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2 symptoms that are uncommon in pulmonary HTN |
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Definition
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gold standard for diagnosing pulmonary HTN |
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Definition
right heart catheterization to measure PA pressure |
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Term
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Definition
JVD, ascites, edema, hepatomegaly, RV heave, tricuspid regurge, loud P2, fixed S2 split, elevated BNPs |
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Term
what treatment is recommended for all groups? |
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Definition
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treatments that improve survival (4) |
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Definition
warfarin, O2, Ca++ channel blockers in responders, epoprostenol |
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Term
what defines a "responder" |
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Definition
administer adenosine, epoprostenol or NO; pt is a responder if mean PA drops more than 10mmHg to less than 40mmHg |
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Ca++ channel blockers used to tx responders (3) |
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Definition
diltiazem, nifedipine, amlodipine |
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Definition
epoprostenol, treprostinil, ilioprost |
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Definition
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Definition
non selective endothelial receptor antagonist |
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