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Pulmonary Hypertension Associated with Respiratory Diseases

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PanVascular Medicine

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Abstract

Pulmonary hypertension is a common complication of many chronic respiratory diseases. The mechanisms contributing to the development of pulmonary hypertension in this context are multiple and go beyond chronic hypoxic vasoconstriction. Outcomes are universally worsened when pulmonary hypertension complicates chronic respiratory disease. Treatment strategies target the underlying disease, as pulmonary vasodilator therapy has proven largely ineffective in trials to date. This chapter summarizes current understanding of the epidemiology, pathophysiology, and treatment of pulmonary hypertension as it relates to a spectrum of chronic respiratory diseases.

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Abbreviations

6MWD:

6-min walking distance

CO2 :

Carbon dioxide

COPD:

Chronic obstructive pulmonary disease

CPET:

Cardiopulmonary exercise testing

CPFE:

Combined pulmonary fibrosis and emphysema

CTD:

Connective tissue disease

DLCO :

Diffusing capacity of the lung for carbon monoxide

ERA:

Endothelin receptor antagonist

FEV1 :

Forced expiratory volume in 1 s

FVC:

Forced vital capacity

HP:

Hypersensitivity pneumonitis

IIP:

Idiopathic interstitial pneumonia

ILD:

Interstitial lung disease

IPF:

Idiopathic pulmonary fibrosis

LAM:

Lymphangioleiomyomatosis

LTOT:

Long-term oxygen therapy

LV:

Left ventricle

LVRS:

Lung volume reduction surgery

NF:

Neurofibromatosis

NO:

Nitric oxide

NSIP:

Nonspecific interstitial pneumonia

NYHA:

New York Heart Association

PA:

Pulmonary artery

PAH:

Pulmonary arterial hypertension

PaO2 :

Arterial partial pressure of oxygen

PASP:

Pulmonary artery systolic pressure

PDE5:

Phosphodiesterase-5

PH:

Pulmonary hypertension

PLCH:

Pulmonary Langerhans cell histiocytosis

PM/DM:

Polymyositis/dermatomyositis

PVOD:

Pulmonary veno-occlusive disease

PVR:

Pulmonary vascular resistance

RV:

Right ventricle

RVSP:

Right ventricular systolic pressure

SLE:

Systemic lupus erythematosus

SS:

Sjogren’s syndrome

SSc:

Systemic sclerosis

TAPSE:

Tricuspid annular plane systolic excursion

TGF-β RII:

Transforming growth factor beta receptor II

TGF-β:

Transforming growth factor beta

TSC:

Tuberous sclerosis

UIP:

Usual interstitial pneumonia

V/Q:

Ventilation/perfusion

VEGF:

Vascular endothelial growth factor

WHO:

World Health Organization

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Authors and Affiliations

  1. Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, 1830 E. Monument Street, 5th Floor, Baltimore, MD, 21205, USA

    Todd M. Kolb M.D., Ph.D. & Prof. Paul M. Hassoun M.D.

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  1. Todd M. Kolb M.D., Ph.D.
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  2. Prof. Paul M. Hassoun M.D.
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Correspondence to Todd M. Kolb M.D., Ph.D. .

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  1. Akademisches Lehrkrankenhaus MLU, Bitterfeld-Wolfen, Germany

    Peter Lanzer

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Kolb, T.M., Hassoun, P.M. (2014). Pulmonary Hypertension Associated with Respiratory Diseases. In: Lanzer, P. (eds) PanVascular Medicine. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-37393-0_207-1

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