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Epidemiology and Disease Classification of Pulmonary Hypertension

  • Chapter
Diagnosis and Management of Pulmonary Hypertension

Part of the book series: Respiratory Medicine ((RM,volume 12))

Abstract

Numerous conditions lead to a sustained increase in pulmonary arterial pressure. Pulmonary hypertension is defined as an elevation of mean pulmonary arterial pressure to 25 mmHg or greater. Currently, the pulmonary hypertensive diseases are organized into five groups that include (1) pulmonary arterial hypertension, (2) pulmonary hypertension owing to left-sided heart disease, (3) pulmonary hypertension owing to chronic lung disease, (4) chronic thromboembolic pulmonary hypertension, and (5) miscellaneous causes. Group 2 and 3 represent the most common causes of pulmonary hypertension. Group 1 (PAH) is exceedingly rare but normally progresses to severe pulmonary hypertension and right ventricular failure. PAH is seen more frequently in patients with connective tissue disease, HIV infection, portal hypertension, congenital cardiac shunts, and those who have used amphetamine-like drugs. This chapter provides an in-depth description of the various pulmonary hypertensive diseases and an overview of their epidemiology, including demographics, risk factors, and distinguishing characteristics.

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Abbreviations

BMPR2:

Bone morphogenic protein receptor-2

CHD:

Congenital heart disease

CTD:

Connective tissue disease

IPAH:

Idiopathic pulmonary arterial hypertension

mPAP:

Mean pulmonary artery pressure

NIH:

National Institutes of Health

PA:

Pulmonary artery

PAH:

Pulmonary arterial hypertension

PAOP:

Pulmonary artery occlusion pressure

PAP:

Pulmonary artery pressure

RHC:

Right heart catheterization

Sch-PAH:

Schistosomiasis associated pulmonary arterial hypertension

TGF-β:

Transforming growth factor beta

WHO:

World Health Organization

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Author information

Authors and Affiliations

  1. Pulmonary Department, Heart Institute, University of Sao Paulo Medical School, Sao Paulo, Brazil

    Rogerio Souza M.D. & Carlos Jardim M.D.

  2. Service de Pneumologie, Hopital Bicetre, Université Paris-Sud, 78 rue du Général Leclerc, Le Kremlin-Bicetre, 94270, Paris, France

    Marc Humbert M.D.

Authors
  1. Rogerio Souza M.D.
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  2. Carlos Jardim M.D.
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  3. Marc Humbert M.D.
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Corresponding author

Correspondence to Marc Humbert M.D. .

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Editors and Affiliations

  1. Division of Pulmonary, Sleep and Critical Care Medicine, Rhode Island Hospital, Alpert Medical School of Brown University, Providence, Rhode Island, USA

    James R. Klinger

  2. Department of Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota, USA

    Robert P. Frantz

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Souza, R., Jardim, C., Humbert, M. (2015). Epidemiology and Disease Classification of Pulmonary Hypertension. In: Klinger, J., Frantz, R. (eds) Diagnosis and Management of Pulmonary Hypertension. Respiratory Medicine, vol 12. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-2636-7_2

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  • DOI: https://doi.org/10.1007/978-1-4939-2636-7_2

  • Publisher Name: Humana Press, New York, NY

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  • Online ISBN: 978-1-4939-2636-7

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