How do we measure pathology in PAH (lung and RV) and what does it tell us about the disease

Abstract

The current understanding of the pathology that underlies pulmonary vascular and right ventricular remodeling in pulmonary hypertension is discussed. Although recent studies underscored the importance of intima and media remodeling and, for the first time, the relevance of perivascular inflammation, much is needed to move the field forward. Reassessment of distribution and extension of the different vascular lesions requires state-of-the-art stereological tools, allied to three-dimensional casting and integration with data concerning cellular and molecular pathobiological processes. This integrated approach is ever more pressing in the right ventricle, because our understanding of key structural alterations of the failing right ventricle in pulmonary hypertension is lacking. This enterprise will enable better translation of pathogenetic processes to the human disease and provide key data to guide diagnostic and prognostic imaging approaches.