Rheumatic heart disease (RHD) and its complications are major health problems worldwide, especially in developing countries, owing to their high prevalence. Mitral stenosis (MS) is one of the most common lesions in RHD, either isolated or in combination with mitral regurgitation, and eventually leads to atrial fibrillation (AF), congestive heart failure, pulmonary hypertension (PH), and other complications, including ischemic stroke or limb ischemia, if not promptly diagnosed and treated. Recent studies have suggested that MS affects the cardiovascular system beyond mere obstructions. The presence of MS in RHD causes significant changes in the cardiovascular system, both upstream and downstream, affecting both the left and right ventricles. Rheumatic MS causes significant structural changes through inflammatory pathways and hemodynamic changes, owing to its obstructive effects. This review aims to discuss the vast changes in the cardiovascular system caused by rheumatic MS. © 2025 by the authors.