Pulmonary hypertension due to left heart disease (PH-LHD) is the most common form of PH affecting approximately 1% of adults worldwide. Initially considered as just a passive consequence of increased pressure in the left chambers, new evidence shows the potential role of genetics, biological factors, or left atrial function. Along with the hemodynamic study, cardiac imaging techniques are essential to advance in the characterization of patients and select therapeutic strategies. Orphan of pharmacological treatments, new therapeutic targets are currently under investigation. The current review aims to review novelties in the fields of pathophysiology, diagnosis, and treatment in this entity.