Right ventricular fibrosis and dysfunction: Actual concepts and common misconceptions

Abstract Fibrosis and remodeling of the right ventricle (RV) are associated with RV dysfunction and mortality of patients with pulmonary hypertension (PH) but it is unknown how much RV fibrosis contributes to RV dysfunction and mortality. RV fibrosis manifests as fibroblast accumulation and collagen deposition which may be excessive. Although extracellular matrix deposition leads to elevated ventricular stiffness, it is not known to which extent it affects RV function. Various animal models of pulmonary hypertension have been established to investigate the role of fibrosis in RV dysfunction and failure. However, they do not perfectly resemble the human disease. In the current review we describe the major characteristics of RV fibrosis, molecular mechanisms regulating the fibrotic process, and discuss how therapeutic targeting of fibrosis might affect RV function.