One More Thought…• …most of <strong>the</strong>se patients are young women in <strong>the</strong>prime of life with no previous health concerns.– Affect women (1.7:1) in <strong>the</strong> 3 rd to 4 th decade of life– Have limited lifespan after diagnosis (mean = 2.5 years)• Specialty centers for treatment• Is anyone dealing with <strong>the</strong> psychosocial and spiritualneeds of <strong>the</strong>se women?– Social Work– Case Managers– Home Healthcare– Pastoral Services– Support Groups
References• Barnett, C. F., Hsue, P. Y., Machado, R. F. (2008). <strong>Pulmonary</strong> hypertension: An increasinglyrecognized complication of hereditary hemolytic anemias and HIV infection. Journal of <strong>the</strong>American Medical Association, 299(3), pp. 324‐331.• Brenot, F., Herve, P., Petitpretz, P., Parent, F., Duroux, P., Simonneau, G. (1993). Primarypulmonary hypertension and fenfluramine use. British Heart Journal, 70(6), pp. 537‐541.• Butler, J., Stankewicz, M. A., Wu, J., Chomsky, D. B., Howser, R. L, Khadim, G. et al. (2005). Pretransplantreversible pulmonary hypertension predicts higher risk for mortality after cardiactransplantation. Journal of Heart and Lung Transplantation, 24(2), pp. 170‐177.• Cheever, K. H. (2005). An overview of pulmonary arterial hypertension: Risks, pathogenesis,clinical manifestations and management. Journal of Cardiovascular Nursing, 20(2), pp. 108‐116.• Chin, K. M., Channick, R. N. & Rubin, U. (2006). Is methamphetamine use associated withidiopathic pulmonary arterial hypertension? Chest, 130(6), pp. 1657‐1663.• Dorfmuller, P., Humbert, M., Perros, F., Sanchez, O., Simonneau, G., Muller, K. M. (2007). Fibrousremodeling of <strong>the</strong> pulmonary venous system in pulmonary arterial hypertension associated withconnective tissue diseases. Human Pathology, 38(6), pp. 893‐902.• Enriquez‐Sarano, M., Rossi, A., Seward, J. B., Bailey, K. R. & Tajik, A. J. (1997). Determinants ofpulmonary hypertension in left ventricular dysfunction. Journal of <strong>the</strong> American College ofCardiology, 29(1), pp. 153‐159.• Hargett, C. W. & Tapson, V. F. (2005). Brain natriuretic peptide: diagnostic and <strong>the</strong>rapeuticimplications in pulmonary arterial hypertension. Seminars in R espiratory and Critical CareMedicine, 26(4), pp. 385‐393.
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Pulmonary Hypertension:Beyond the S
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What is the Big Deal?• Patients w
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Independent Predictor of Mortality
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Etiology• Noxious stimuli exposur
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Components
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Pathways Implicated in PHTNTXA2Pre
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Components• Prostacyclin- Prostag
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Abnormalities in CoagulationPlatele
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Pathophysiology• Increased flow-
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Pathophysiology• Low resistance,
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Pathophysiology ProgressionDeath
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ClassificationARTERIAL• Idopathic
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ARTERIAL HYPERTENSION
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Pulmonary Arterial Hypertension•
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Collagen Vascular Disease• Sclero
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Portopulmonary HTN• Portal HTN +
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Methamphetamine• Amphetamine, met
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VENOUS HYPERTENSION
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Left Heart Disease• LV systolic d
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LV Pressure‐Volume Curvehttp://ww
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Congenital Heart Disease• Systemi
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PHTN associated with lungdisease/hy
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Thromboembolic Disease• Thromboem
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Miscellaneous• Sarcoidosis• His
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SYMPTOMSAnd then I felttired…
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Signs and Symptoms• Dyspnea- Most
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Physical Exam• Prominent, split,
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ECG Changes• Right‐axis deviati
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CXRwww.emedicine.com/RADIO/topic583
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V/Q Scanwww.emedicine/RADIO/topic58
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Echohttp://www.emedicine.com/med/im
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DIAGNOSIS
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- Page 72 and 73: Nocturnal Desaturationwww.rnceus.co
- Page 74 and 75: Digoxin• Controversial- No mortal
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- Page 78 and 79: Treatment For LV sided Heart Diseas
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- Page 82 and 83: Survival and Prostacyclin• 162 pa
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- Page 90 and 91: Endothelin Receptor Antagonists•
- Page 92 and 93: Nitric Oxide• Increases intracell
- Page 94 and 95: Nitric Oxide• Vascular effects- D
- Page 96 and 97: Weaning Nitric Oxide• Case Study
- Page 98 and 99: Surgical Options• Pulmonary Endar
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- Page 102 and 103: FUTURE THERAPIES
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- Page 106 and 107: Serotonin Antagonists• “Treatme
- Page 108 and 109: More Hope…• Perhaps the inhibit
- Page 110 and 111: You knew this next one was coming
- Page 112 and 113: CONCLUSIONSThe End
- Page 114 and 115: WorkUp• Autoantibody tests, HIV t
- Page 118 and 119: References• Herve, P., Launay, J.
- Page 120: References• Nagaya, N., Nishikimi