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Pulmonary Hypertension Associated with HIV, Liver Disease, Sarcoidosis, and Sickle Cell Disease

  • Chapter
Pulmonary Hypertension

Part of the book series: Contemporary Cardiology™ ((CONCARD))

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Abstract

HIV, portopulmonary hypertension, sarcoidosis, and sickle cell disease (SCD) are all associated with PH. Although specific histological features of the pulmonary vasculature may differ among the various entities, they share a number of similarities. The PH manifests itself in a minority of these patients, consistent with the idea that gene mutations may predispose certain individuals to developing PH, probably in combination with other factors. Multiple factors seem to contribute, including endothelial dysfunction, vasodilator/vasoconstrictor mediator imbalance, immunological and oxidant mechanisms, and structural abnormalities of the vasculature related to injected foreign material (mainly in HIV and some SCD patients) and to parenchymal destruction (particularly in sarcoidosis and SCD). In addition, some entities, such as portopulmonary hypertension and SCD, are characterized by relatively high cardiac outputs that may predispose to PH by means of increased shear stress. Patients with SCD not uncommonly have a component of pulmonary venous hypertension related to LV diastolic dysfunction, underscoring the need to perform right heart catheterizations to properly diagnose patients. The PAH in these entities is also often responsive to a variety of medical therapies, although this inference is based mainly on case reports and small cohort studies and not on properly designed randomized trials.

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  1. Kimberly A. Fisher
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  2. Elizabeth S. Klings
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Editors and Affiliations

  1. Division of Pulmonary, Critical Care and Sleep Medicine Tufts Medical Center, Boston, MA

    Nicholas S. Hill MD

  2. Pulmonary Center, Boston University, Boston, MA

    Harrison W. Farber MD

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Fisher, K.A., Klings, E.S. (2008). Pulmonary Hypertension Associated with HIV, Liver Disease, Sarcoidosis, and Sickle Cell Disease. In: Hill, N.S., Farber, H.W. (eds) Pulmonary Hypertension. Contemporary Cardiology™. Humana Press. https://doi.org/10.1007/978-1-60327-075-5_9

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  • DOI: https://doi.org/10.1007/978-1-60327-075-5_9

  • Publisher Name: Humana Press

  • Print ISBN: 978-1-58829-661-0

  • Online ISBN: 978-1-60327-075-5

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