Double orifice mitral valve: rare disease with a wide range of presentation

Transcatheter Edge-to-Edge Repair for Severe Mitral Regurgitation With Double-Orifice Mitral Valve

“…Patients with DOMV can be asymptomatic or have symptoms of valvular dysfunction if they are not associated with other malformations. 1 One-half of patients with DOMV have valvular dysfunction, including mitral stenosis or MR. 2 In this case, MR was located in the small ALO, secondary to ruptured chordae tendineae. This anatomic structure made the patient very fortunate to be treated with TEER because the remaining posteromedial foramen was large enough to avoid postoperative mitral stenosis.…”

Role of 3D transoesophageal echocardiography in correct diagnosis of congenital isolated double orifice mitral valve in a young adult misdiagnosed as rheumatic heart disease

“…The management of DOMV depends on the patient’s symptoms, lesions of the MV and any associated valvular or structural abnormalities. DOMV can present as stenosis or regurgitation, and depending on its severity and patient’s symptoms, MV repair or replacement may be needed 8…”

“…Due to its rarity and a lack of awareness regarding its echocardiographic characteristics, the diagnosis of DOMV can be challenging and may be mistaken for other conditions, such as mitral stenosis, mitral regurgitation or MV prolapse 8. On TTE, apart from the restricted motion of MV leaflets in the parasternal long-axis view (PLAX), there was no other evidence to suggest rheumatic involvement of the MV, in the form of subvalvular thickening, commissural fusion, chordal shortening, etc.…”