Double orifice mitral valve (DOMV) is a rare congenital anomaly that is often associated with cardiac malformation. Valve dysfunction usually presents in childhood; therefore, most cases are diagnosed with DOMV in childhood. Its prevalence and prognostic relevance in adulthood are unknown. Here, we report a case of a 38-year-old woman who presented to the outpatient clinic with an abnormal electrocardiogram and was found to have an isolated double orifice mitral valve malformation on transthoracic echocardiography. Echocardiography is the diagnostic tool of choice for patients with double orifice mitral valves. We should familiarize ourselves with the echocardiographic features of DOMV in order to improve the detection.

Double orifice mitral valve (DOMV) is a rare congenital valve malformation. Due to the insufficient understanding and awareness of its echocardiographic characteristics, there is a risk of misdiagnosis. Here, we report a case of echocardiographic diagnosis of congenital DOMV malformation in a 56-year-old male patient who visited our hospital for systematic physical examination, during which DOMV was not diagnosed. The following year, the patient visited our hospital again, and echocardiography showed two approximately circular orifices on the short axis view of the mitral valve. During diastole, two red Doppler blood flow signals were observed entering the left ventricle simultaneously. A small amount of blue Doppler signal revealed reflux into the left atrium during systole, leading to the diagnose of congenital heart disease with DOMV malformation, without other cardiac structural abnormality. Because the patient did not have obvious discomfort, he did not receive any special treatment and is regularly followed-up.

UNASSIGNED: A double orifice mitral valve (DOMV) represents a rare congenital malformation characterized by two valve orifices with two separate subvalvular apparatus. Double orifice mitral valve is congenital anomaly of the subvalvular mitral valve apparatus consisting of an accessory bridge of fibrous tissue, which partially or completely divides the mitral valve into two orifices.
UNASSIGNED: A 30-year young male presented with dyspnoea and palpitation for 4 years, joint pain for 2 years and weakness of right upper limb and lower limb for 6 months. On clinical examination, Boutonniere, Swan neck, and Z-deformity of hand and foot metatarsal bone deformities are noted, on further evaluation, patient was diagnosed as a case of DOMV and was managed conservatively since patient was not willing for surgery.
UNASSIGNED: Two-dimensional echocardiography is the best detection method, the parasternal short-axis view being most useful to show DOMV.

Double-orifice mitral valve (DOMV) is an unusual congenital anomaly characterized by a mitral valve with a single fibrous annulus with two orifices or rarely two orifices with two separate mitral annuli opening into the left ventricle. We present a first report of a patient with a DOMV with supramitral ring (SMR), subaortic membrane (SAM), a large ventricular septal defect (VSD) with more than 50% aortic override, and severe pulmonary arterial hypertrophy (PAH). This patient underwent excision of the SAM, and SMR, with closure of the VSD together under cardiopulmonary bypass (CPB). However postoperatively, the patient developed an irreversible fatal pulmonary hypertensive crisis (PHC), immediately after transferring the patient to the cardiac intensive care unit from the operating room (OR). The PHC was refractory to intravenous and inhaled milrinone and nitroglycerine and intravenous adrenaline, dobutamine, norepinephrine, vasopressin, patent foramen oval (PFO), and CPB support. The management of DOMV and perioperative pulmonary hypertension is discussed.

A 24-year-old female patient presented to us with progressive dyspnea on exertion for last three year. She was not a known case of rheumatic heart disease. Her physical examination showed regular pulse and her blood pressure was 100/76 mm Hg. Cardiac palpation showed grade 3 parasternal heave and auscultation revelled an accentuated first heart sound, loud P2 and mid-diastolic long rumbling murmur at apex and pansystolic murmur of tricuspid regurgitation at lower left sterna border. Chest X-ray showed evidence of grade 3 pulmonary venous congestion. Transthoracic and transesophageal two-dimensional echocardiography revealed a double-orifice mitral valve of complete bridge type at the leaflet level. Both orifice sizes were unequal, with the anterolateral orifice being smaller than its counterpart. There was moderate subvalvular fusion and both commisures were fused. Color doppler examination showed two separate mitral diastolic flows with mean gradients of 22 mm and 20 mm of Hg, respectively. There was no mitral regurgitation and no left atrial or appendage clot was seen by transesophageal echocardiography. Transseptal puncture was done by the modified fluoroscopic method. Posteromedial orifice was crossed with a 24 mm Inoue balloon and dilated using the stepwise dilation technique. Anterolateral orifice was not crossed by Inuoe balloon after multiple attempts. A TYSHAK (NuMAD Canada Inc.) balloon (16 × 40mm) was taken over the wire and inflated successfully across the anterolateral orifice with the help of transthoracic echocardiography guidance. Mean gradient become 9 and 8 mm across the medial and lateral orifice. Patient was discharged in stable condition after two day. © 2015 Wiley Periodicals, Inc.

Double orifice mitral valve (DOMV) is an uncommon anomaly characterized by a mitral valve with a single fibrous annulus with two orifices opening into the left ventricle. Here we present a rare case of DOMV with ventricular septal defect (VSD) which was detected by 2-D echocardiography.

Double orifice mitral valve (DOMV) is an uncommon anomaly of surgical importance characterized by a mitral valve with a single fibrous annulus with two orifices opening into the left ventricle (LV). Subvalvular structures, especially the tensor apparatus, invariably show various degrees of abnormality. Associated congenital heart defects are common, though DOMV can occur as an isolated anomaly. Two-dimensional echocardiography is useful for diagnosis but combining it with real-time three-dimensional echocardiography helps in a more detailed evaluation of mitral valve and subvalvular structures as is shown in this case description.