phtn - Pulmonary hypertension Current perspectives in the...

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Pulmonary hypertension Current perspectives in the diagnosis and management Chandana Senior Resident, Dept. of Pulmonary medicine PGIMER
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Definition Classification Pathobiology Diagnosis Treatment
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Pulmonary artery hypertension PPH Sporadic Familial Related to: Collagen vascular disease Congenital systemic to pulmonary shunts Portal hypertension HIV infection Drugs/toxins Persistent PH of the newborn Pulmonary venous hypertension Left-sided atrial or ventricular heart disease Left-sided valvular heart disease Fibrosing mediastinitis Adenopathy/tumors Pulmonary veno-occlusive disease PH associated with disorders of the respiratory system and/orhypoxemia COPD Interstitial lung disease Sleep-disordered breathing Alveolar hypoventilatory disorders Long-term exposure to high altitude PH due to chronic thrombotic and/or embolic disease Thromboembolic obstruction of proximal pulmonary arteries Pulmonary embolism (thrombus, tumor, ova and/or parasites, foreign material) PH due to disorders directly affecting the pulmonary vasculature Schistosomiasis Sarcoidosis Pulmonary capillary hemangiomatosis EvianNomenclatureandClassification ofPulmonaryHypertension (1998) Diagnostic Classification
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Pathobiology of pulmonary hypertension Pathologic hallmark Vasoconstriction Vascular smooth muscle hypertrophy Vascular Endothelial cell proliferation remodelling Adventitial cell proliferation Vasoconstriction/Vascular smooth muscle cell hypertrophy - Vascular endothelial cell dysfunction - Imbalance between endothelial cell mediators Loss of vasodilators ( NO, PGI2 ) Vasoconstrictors ( TXA2, ET-1, Serotonin) - K+ channel dysfunction Ca++ Vasoconstriction -Hypoxia,5HT, ET I Growth factors for smooth muscle cells
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Pathobiology of pulmonary hypertension - Intermediate cells, pericytes proliferate Neomuscularisation of distal vessels 2) Endothelial cell proliferation/plexiform lesions - VEGF,other growth factors Endothelial cell proliferation - Mutations in BMPR – II gene,5HT transporter Plexiform lesions – Proliferation of endothelial and smooth muscle cells Arterial lumen occlusion,aneurysmal dilatation
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Pathobiology of pulmonary hypertension 3) Extra cellular matrix remodeling/adventitial proliferation - Increased ECM degradation - Elastase,matrix metalloproteiases - Perivenular inflammatory cell infiltrate - Increased – IL 1B, Increased IL-6 4) Insitu thrombosis - Slowing of pulmonary blood flow - Altered expression of PGI2,NO. - Increased PAI,fibrinopeptide A,increased factor VIII C
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Vascular remodelling PAH PH sec. to lung dis. 1) Medial smooth muscle HT ++ ++ 2) Distal small vessel neo musc. ++ ++ 3) Adventitial changes ++ + 4) Intimal proliferation marked mild 5) Monoclonal endoproliferation + - 6) Plexogenic lesions + - 7) Insitu thrombosis common rare
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Evaluation of PHTN PHTN suspected NO S/S CXR,ECG, RVSP>50 w/u RVSP <36 TT Echo TRvel>3.4 etiology TRvel<2.8 RVSP 36-50 >45 PAS TRvel 2.8-3.4 >35 Mpap NYHA 1 NYHA 2-4 Rt heart cath F/U 35-45 <35 25- 35 <25 exercise RVSP/PAS F/U
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This note was uploaded on 12/03/2011 for the course MEDICINE 350 taught by Professor Dr.aslam during the Winter '07 term at Medical College.

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phtn - Pulmonary hypertension Current perspectives in the...

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