Prognosis and outcome

Remodeling of the vessel structure is potentially reversible when caused by mechanical forces. A reduction in blood flow and perfusion pressure also normalizes shear stresses to intact endothelial cells which are essential for reversal of vascular remodeling. The potential recovery of the pulmonary circulation has been demonstrated in many clinical entities, for example after correction of mitral valve disease, pulmonary artery banding in congenital pulmonary hypertension, or closure of cardiac septal defects. Depending on the duration of pulmonary hypertension, it may take years for the vessels to restructure and pulmonary artery pressures to normalize. Involvement of the right and left ventricles in the disease process must also be considered as this will influence or limit restoration of a normal pulmonary circulation.

In contrast with chronic pulmonary hypertension resulting from increased blood flow or high pressures, remodeling of pulmonary vasculature is almost irreversible in patients when the disease process results from chronic inflammatory processes, immunological alterations, or thrombogenic obliteration of the lumen. Interstitial fibrosis and destruction of the regular histological pattern, including loss of endothelial cell integrity, determines the extent of future deterioration.

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