Understanding the Pathobiology of Pulmonary Vascular Disease



Pulmonary arterial hypertension (PAH) is a panvasculopathy that results in a progressive increase in pulmonary arterial pressure in association with variable degrees of pulmonary vascular remodeling, vasoconstriction, and in situ thrombosis. This leads to concentric obliteration of the pulmonary arteriole and the development of plexiform lesions that in turn result in an increased pulmonary vascular resistance and eventual right heart failure and death. The complex pathological features of pulmonary arterial hypertension (PAH) are highlighted in this review. A greater understanding of this complex pathobiology of PAH is essential for the future development of new therapeutic options.


Pulmonary Hypertension Pulmonary Arterial Hypertension Right Ventricle Vasoactive Intestinal Peptide Pulmonary Vascular Resistance 



Figure 13.1 courtesy of Ellen Reimer, M.D., J.D., Assistant Professor of Pathology and Laboratory Medicine, Medical University of South Carolina.

Figures 13.2 and 13.3 courtesy of Russel Harley, M.D., Professor of Pathology and Laboratory Medicine, MUS and Chairman, Dept. of Pulmonary and Mediastinal Pathology, AFIP.

DisclosuresDr. Highland is on the speaker’s bureau and/or has grants/contracts with Actelion Pharmaceuticals, Gilead Sciences, and Pfizer Inc.


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© Springer-Verlag London Limited 2012

Authors and Affiliations

  1. 1.Pulmonary Hypertension ProgramMedical University of South CarolinaCharlestonUSA

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