Double-Orifice Mitral Stenosis of Rheumatic Origin: A Case Report and Literature Review

Abstract

Double-orifice mitral valve (DOMV) is an uncommon anatomical anomaly, which may be either congenital or acquired. In rheumatic heart disease, subvalvular fusion and leaflet remodeling can mimic a DOMV appearance, complicating the differential diagnosis. We present the case of a 40-year-old woman with severe DOMV mitral stenosis of rheumatic origin, confirmed histopathologically after mitral valve replacement. Transthoracic echocardiography revealed two distinct mitral orifices with commissural fusion, each with a valve area of 0.7 cm². The mean transmitral gradient was 14 mmHg, with pulmonary artery systolic pressure estimated at 55 mmHg. Given the high risk of leaflet rupture with percutaneous balloon mitral valvotomy, surgical valve replacement was performed, combined with a single-vessel coronary bypass. Postoperative recovery was uneventful. This case emphasizes the importance of distinguishing congenital from acquired DOMV, as management and prognosis differ significantly. Advances in three-dimensional echocardiography and surgical outcomes in DOMV are also discussed.