Pulmonary hypertension – disease mechanisms

Česká verzia

Authors: Martin Helán ^1,2; Anna Konieczna ^2,3; Martin Klabusay ^2,3; Vladimír Šrámek ¹
Authors place of work: Anesteziologickoresuscitační klinika LF MU a FN u sv. Anny Brno, přednosta doc. MUDr. Vladimír Šrámek, Ph. D. ¹; Mezinárodní centrum klinického výzkumu FN u sv. Anny Brno, ředitel Gorazd B. Stokin, M. D., MSc., Ph. D. ²; LF UP Olomouc, děkan prof. MUDr. Milan Kolář, Ph. D. ³
Published in the journal: Vnitř Lék 2014; 60(10): 852-858
Category: Reviews

Summary

Pulmonary hypertension (PH) is known for its variable etiology. PH pathophysiology is very complex and our therapeutic options are limited. Most of known underlying disease mechanisms play a role across all etiological groups of PH, and they are followed by the same morphological and functional changes of pulmonary vasculature. Mostly, we are not able to determine whether one particular mechanism works as a cause or consequence in the chain of events. An imbalance between vasoconstriction and vasodilation becomes the major functional change of pulmonary vasculature in PH. The main morphological changes (termed together as “remodeling”) include cell hyperplasia of pulmonary artery leading to its thickening and narrowing, and impaired regulation of extracellular matrix production leading to reduction in its elasticity. As a result of all these changes, the peripheral vascular resistance in pulmonary vascular bed rises, thus increasing afterload of the right ventricle and finally progressing to its failure. This review aims to summarize and explain the nature of the functional and histological changes in pulmonary arteries which occur in pulmonary hypertension, separately define the role of endothelium and pulmonary artery myocytes, and discuss the most important known pathophysiological mechanisms that lead to these changes.

Key words:
endothelium – intracellular calcium signaling – nitric oxide – pulmonary artery – pulmonary hypertension – remodeling – smooth muscle cell

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