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02 Pulmonary Hypertension
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Published
06/26/2023
What is pulmonary hypertension:Group of pulmonary diseases of pulmonary vasculature resulting from increased pressure in the {{c1::pulmonary circulati…
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Normal mPAP at rest is {{c1::14}} +/- 3 mmHg with upper limit of normal being {{c1::20}}
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Definition of pulmonary htn: based on {{c1::R heart catherterization}}{{c1::>/= 20 }} mmHgCommonly acessed via the external jugular, femoral vein
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WHO divides pulmonary htn into {{c1::5 }} groupsGroup 1 is sinister, but more treatable. Problem is in the {{c2::blood vessels themselves}}Group …
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Pulmonary HTN vs Pulmonary arterial HTN:PH- mPAP >/= {{c1::20}}mmHGPAH- mPAP >/= {{c1::20}}mmhg + {{c2::PAWP}} </= {{c3::14}}mmHg + {{c2::PVR…
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{{c1::Core Pulmonale}}:An alteration in the structure and fxn of the r ventricle of the heart caused by a primary disorder of the respiratory system (…
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PAH: Epidmeiology:Rare disease- incidence and prevalence are difficult to estimateMore common in {{c1::women}}Mean age is approximately {{c1::50}…
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PAH Epidemiology: {{c1::Shistosomiasis}}:{{c1::Fluke bores}} into pulmonary circulation from hepatic invasion and casues significant imflammation- mor…
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Pulmonary Arterial Hypertension: WHO 1:Major cause of PAH in united States: {{c1::Autoimmune disease}}
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PAH: Heritable {{c1::BMPR2 }}Over 300 mutations have been identifiedFound in > 70% of pts with H-PAH
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PAH: Drug induced:Definitive{{c1::Aminorex}}{{c1::Fenfluramine}}Dexfenluramine{{c1::Toxic rapeseed}} oil Benfluorex{{c2::SSRI}} reuptakeLikely{{c…
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PAH: Connective Tissue Disease: {{c1::Scleroderma}}Most prevalent and studied PAH associated with CTDPoorer prognosis than other types of PAHPAH …
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Prognosis of PAH → {{c1::Progressive}} Disease, we only tx {{c1::sx.}}
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Characterization of pulmonary Arterial Hypertension: Characterized by pathologic changes to pulmonary arteries{{c1::Vasoconstriction}}, {{c1::vas…
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PAH → Clinical Presentation:{{c1::Dyspnea on exertion}}- most common presentation{{c1::Fatigue}}Chest pain / discomfort EdemaPhysical Finding:&nb…
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Physiologic Assessments in PAH: Chest Xray:Abnormal at diagnosis of 90% of IPAHFindings: Central Pulmonary {{c2::arterial dilation}}Prining …
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Echo: >{{c1::2.9}} - probably PAH<{{c1::2.5}} - rules it out
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PAH Dx: {{c2::Right heart Cath}}:Required to establish and confirm PAH and Dx PAH is defined by elevated mPap >/= {{c1::20}} mmHgWith PCWP <…
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PAH Tx:Currently {{c1::no cure for PAH}}Modern Advanced PAH therapies can markedly improve patiets sx and slow rate of deterioration
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PAH tx Goals:Improve functional classImprove {{c1::exercise capacity}}Improve {{c1::QOL}}Delay in clinical worseningDecreased hospitalization Imp…
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PAH: General And supportive Measures:Early referral to {{c1::expert center}}- {{c1::BEtter prognosis if sends to specialist who commonly deals wi…
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PAH patients must be aware of {{c1::high altitude}} environment
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Conventional / Supportive Therapy: Provide sx benefit:Supplement {{c1::o2}}- keep o2 {{c1::>90%}}Oral {{c1::anticoagulants}}{{c2::Diuretics}}{{c2::…
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PAH: Prostacyclin Pathway:{{c1::Espoprostenol}}- OldestContinuous {{c1::IV Inhalation}}20-40 ng/kg/minClass Indicated: {{c4::III, IV}}Only PAH- Clinic…
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PAH Therapy: {{c1::NO}} Pathway:{{c2::Sildenafil}}, {{c2::Tadalafil}}, {{c2::Riociguat}}All indicated for class {{c1::II, II, IV}}
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PAH: Endothelin Pathway: {{c2::Bosetan}}- oldest- must monitor lfts{{c1::Ambrisentan}}:First line{{c1::Macitentan}}First line{{c2::Enhanced tissue pen…
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