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Pulmonary Vascular Disease
Downstate > MS2 Respiratory Block > Pulmonary HTN and Drugs
39
Medical
Graduate
02/10/2011

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Cards

Term
normal mean PA pressure
Definition
9-16mmHg
Term
normal LA pressure
Definition
2-12mmHg
Term
definition of pulmonary HTN
Definition
mean PA pressure >25mmHg at rest or >30mmHg at exercise
Term
pre-capillary causes of pulmonary HTN (4)
Definition
diseases affecting pulmonary aa., lung diseases, external compression, hypoventilation
Term
post-capillary causes of pulmonary HTN (2)
Definition
left heart disease, veno-occlusive disease
Term
PWCP - PA > 10mmHg -> pre or post capillary?
Definition
pre-capillary
Term
PWCP - PA < 5mmHg -> pre or post capillary?
Definition
post--specifically heart disease
Term
PWCP - PA = normal -> pre or post capillary?
Definition
post--specifically, veno-occlusive disease
Term
10% of pulmonary HTN cases; caused by defect in BMPR2 gene; dx?
Definition
familial pulmonary HTN
Term
epidemiology of primary pulmonary HTN
Definition
F>M, 30-40yo, very rare
Term
most common causes of pulmonary HTN (2)
Definition
COPD, left heart disease
Term
how do kyphoscoliosis and sleep apnea cause pulmonary HTN?
Definition
hypoventilation
Term
histological characteristics of idiopathic pulmonary HTN (4)
Definition
plexiform lesions, medial hypertrophy, intimal hyperplasia and fibrosis of medium-size aa.
Term
when BMPR2 is not the cause, what pathogeneses contribute to pulmonary HTN? (3)
Definition
endothelial dysfunction, dysfunctional K+ channels, vasoactive amines
Term
which endothelin-1 receptor causes vasoconstriction? vasodilation?
Definition
ETA -> vasoconstriction; ETB -> vasodilation
Term
how does K+ channel dysfunction cause pulmonary HTN?
Definition
K+ channel dysfunction -> increase intracellular Ca++ -> increase vascular tone
Term
what factors increase pulmonary a. pressure? (3)
Definition
increased CO (volume/pressure overload), increased PVR (obstruction, vasoconstriction), and increase LA pressure (left heart disease)
Term
how does left-to-right shunt contribute to pulmonary HTN?
Definition
volume overload
Term
how does a large LA myxoma contribute to pulmonary hypertension?
Definition
pressure overload
Term
how does COPD, high altitude, and hypoventilation contribute to pulmonary HTN?
Definition
reflex vasoconstriction
Term
diseases that obliterate pulmonary vasculature (4)
Definition
thrombo-embolism, vasculitis, emphysema, extrinsic compression
Term
group 1
Definition
primary, familial, congenital, portal HTN, infection; always a candidate for advanced therapy
Term
group 2
Definition
left heart disease; always work up for thrombo-embolic disease
Term
group 3
Definition
lung disease
Term
group 4
Definition
thrombo-embolic disease; dx via V/Q scan
Term
pt presents with pulmonary HTN, vasculitis, skin lesions, low DLCO but normal PFTs; what group does this belong in?
Definition
group 5
Term
2 symptoms that are uncommon in pulmonary HTN
Definition
cough, hemoptysis
Term
gold standard for diagnosing pulmonary HTN
Definition
right heart catheterization to measure PA pressure
Term
signs of RHF
Definition
JVD, ascites, edema, hepatomegaly, RV heave, tricuspid regurge, loud P2, fixed S2 split, elevated BNPs
Term
what treatment is recommended for all groups?
Definition
oxygen, anticoagulation
Term
treatments that improve survival (4)
Definition
warfarin, O2, Ca++ channel blockers in responders, epoprostenol
Term
what defines a "responder"
Definition
administer adenosine, epoprostenol or NO; pt is a responder if mean PA drops more than 10mmHg to less than 40mmHg
Term
Ca++ channel blockers used to tx responders (3)
Definition
diltiazem, nifedipine, amlodipine
Term
prostacyclin analogs (3)
Definition
epoprostenol, treprostinil, ilioprost
Term
prostanoid given subQ
Definition
treprostanil
Term
inhaled prostanoid
Definition
ilioprost
Term
bosentan
Definition
non selective endothelial receptor antagonist
Term
ETA antagonist
Definition
ambrisentan
Term
inhibits PDE
Definition
sildenafil
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