Pulmonary Arterial Hypertension Flashcards
What is the formula for Pulmonary Vascular Resistance?
PVR=(mPAP - PAWP)/ CO
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What procedure is done to diagnose, classify and devlop treatment for Pulmonary Artery HTN?
Right Heart Catheterization
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mPAP can be increased by what four mechanisms?
- Elevated resistance to blood flow within arterial circulation
- Increased pulmonary venous pressure from Left heart disease
- Chronically increased pulmonary blood flow
- A combination of these processes
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PH can result from abnormalities in the __________________ or ________________ components of the lung circulation, sometimes includes contributions from ____________.
PH can result from abnormalities in the arterial or venous components of the lung circulation, sometimes includes contributions from both
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After Right heart catheterization the severity of the PH can be determined to be:
Mild PH (mPAP = ________________)
Moderate PH (mPAP = ____________)
Severe PH (mPAP= ________________)
Mild PH (mPAP = 20-30mmHg)
Moderate PH (mPAP = 31-40mmHg)
Severe PH (mPAP= >40mmHg)
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Which diagnostic test or screening tool can be done to estimate pulmonary arterial systolic pressure (PSAP)?
Echocardiogram
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Can a echocardiographic Pulmonary arterial systolic pressure of >41mmHg provide a definitive diagnosis for Pulmonary Hypertention?
**No
Although echocardiographic
PASP > 41 mmHg is relatively sensitive and specific for PH,* it cannot provide the accurate mPAP for definitive diagnosis*
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How much volume can a normal Pulmonary circulation accomodate without a marked change in mean pulmonary artery pressure (mPAP)?
Normal pulmonary circulation can accommodate a fourfold increase in COP without a marked change in mPAP
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What three things can a TTE reveal to help diagnose Pulmonary Artery HTN?
- RA enlargement
- RV enlargement
- Elevated Peak tricuspid-regurgitation
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According to the World Health Organization, PAH is classified as a __________ disease. It affects ________ people per million per year
According to the World Health Organization, PAH is classified as a rare disease effecting15 ppl per million per year
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Does idopathic PAH have any identifiable risk factors?
NOPE
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What medication has shown long-term improvements in 1 in 8 patients with PAH?
Calcium channel blockers
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What percent of patients have the inherited genetic protein mutation (BMPR2) that can cause PAH?
What is BMPR2?
3% of PAH cases are deemed inheritable.
BMPR2 - bone morphogenetic protein receptor type 2
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Remaining cases of PAH that are not genetic mutations are caused by:
a. drugs
b. toxins
c. various diseases
d. all of the above
d. all the above
The remaining cases are designated “associated PAH,” since they can be ascribed to manifestations of drugs, toxins, or other diseases
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Current data shows PAH develops in this age range and population.
Current data shows a demographic shift, now with older pts and more men being diagnosed
PAH was traditionally a disease of young women, with median survival rate of 3 yrs
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Even with improved diagnosis of PAH and therapies the 1 year mortality rate is:
a. 10%
b. 15%
c. 25%
d. 5%
b. 15%
Despite improved diagnosis and therapy, 1-year mortality is** ̴15%**
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What medication has shown long-term improvements in 1 in 8 patients with PAH?
Calcium channel blockers
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What PAH treatment mimics the effect of prostacyclin to produce vasodilation while inhibiting platelet aggregation.
Prostanoids!
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What are the four examples of Prostanoids medications and their routes of administration?
epoprostenol (IV)
iloprost (inhaled)
treprostinil (SQ, IV, INH, PO)
beraprost(PO)
*
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Which Prostanoid medication has been PROVEN to reduce mortality?
Epoprostenol IV
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What is the MOA of Postanoids, when using it to treat pulmonary artery hypertenision?
- mimic the effect of prostacyclin to produce vasodilation while inhibiting platelet aggregation.
- They also have anti-inflammatory effects and may reduce proliferation of vascular smooth muscle cells
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How does Endothilin Receptor antagonists (ERAs) improve pulmonary arterial hypertenion
hemodynamics and exercise capacity
The vascular endothelial dysfunction associated with PAH involves an imbalance between vasodilating (nitric oxide) and vasoconstricting (endothelin) substances. ERAs have been shown to improve hemodynamics and exercise capacity
Slide 37
How does nitric oxide/ guanylate cycase improve pulmonary arterial hypertenion?
nitric oxide produces pulmonary vasodilation by stimulating guanylate cyclase and cGMP formation in smooth muscle cells.
*
This effect is transient because nitric oxide is quickly bound by hgb and degraded by phosphodiesterase type 5*
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What are the areas that nitric oxide has been widely used?
perioperative
critical care
preparpations for home
slide 37
True or False: Chronic therapy has been directed toward PD-5 inhibitors
True
Slide 37
What are the nonspecific signs of pulmonary arterial hypertension for preop consideration?
- fatigue
- dyspnea
- cough
Slide 38
What consideration should be given to pulmonary arterial hypertension procedure with pre-op?
- venous embolism
- elevations in venous and/or airway
- pressure hypoxic pulmonary
Slide 38
What are the more advance signs to take in consideration for preop for patients with pulmonary arterial hypertnsion?
**angina and syncope **
occur with exercise if coronary blood flow cannot meet demand of a hypertrophied RV
Slide 38
Patient with pulmonary arterial hypertension may exhibit____ on physical exam
list
- parasternal lift
- accentuated S2, S3 and/or s4 gallop
- JVD
- peripheral edema
- hepatomegaly
- ascites
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PAH Preop Considerations
What is recommended prior to mod-high risk surgery in pts with mod-severe PH?
Right Heart Cath
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PAH Preop Considerations
- Due to potential discrepancies btw PAWP and LVEDP, a _____ _____ ____ is done with coexisting left heart dz bc may lead to ____ of PH
- Vasoreactivity testing, often with inhaled _____ ______ , during right heart cath to determine responsiveness to ______ therapy
o __-__ % PAH pts are nonresponsive to inhaled nitric oxide, but those that are responsive also respond to ____ ***
- left heart cath
- misclassification
- nitric oxide
- vasodilator
- 85-90%
- CCB’s
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