Perioperative management of a patient with double orifice mitral valve with supramitral ring with subaortic membrane with ventricular septal defect and severe pulmonary hypertension: Report of a rare case

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.

Echocardiographic diagnosis of congenital double orifice of mitral valve deformmity / 中华超声影像学杂志

Objective To discuss the morphological features and diagnosis of congenial double orifice mitral valve (DOMV) deformity by echocardiography.Methods Twenty consecutive patients were examined.The changes of the morphology and flow dynamics of DOMV were studied,and other congenital cardiac abnormalities were also observed.These results were compared with those of surgery.Results Thirteen cases of adult patients were all with significant hemodynamic abnormalities,and underwent mitral replacement.One case of school child with muscular ventricular septal defect underwent mitral replacement because of obvious hemodynamic changes.Six cases were under 2 years old,2 cases with complete endocardial cushion defect accompanied with pulmonary hypertension,1 case with partial endocardial cushion defect accompanied with coarctation of the aorta underwent corrective operation.No significant hemodynamic abnormalities in 2 cases,1 case was associated with coarctation of the aorta and patent ductus arteriosus,the other patient with membranous ventricular septal defect.Severe stenosis in 1 case with papillary muscle dysplasia,because there was no suitable operation and take conservative therapy.Seventeen cases were confirmed by the results of open-heart surgery.Conclusions Echocardiography can provide an accurate method to diagnose DOMV and evaluate the hemodynamic changes.

Results for repair of 28 cases of endocardial cushion defects combined with double-orifice mitral valve / 中华胸心血管外科杂志

Objective To review the surgical results of 28 cases of endecardial cushion defect combined with double-ori-rice mitral valve. Methods Of 860 consecutive patients with endocardial cushion defect, double-orifice mitral valve was iden-tiffed in 28 patients (3.26% ) form October 1996 to November 2007. Intracardiac deformities were corrected simultaneously during the operation. Preoperative mitral valve function, surgical procedures and incidence of pestoperative mitral valve dys-function were reviewed and compared between patients with total endecardial cushion defect ( group Ⅰ, n = 11 ) and partial en-docardial cushion defect ( group Ⅱ, n = 17). Results There were 4 operative deaths in group Ⅰ caused by severe pulmonary inflammation in 3 cases and low cardiac output in 1 case. Two cases were identified as severe mitral valve dysfunction or steao-sis in reoperaticu or autopsy. There was no later death. Mitral valve function is not eatisfactory in group Ⅱ as compared with group Ⅰ in 3 to 89 months of follow-up( averaging 33 months). Conclusion Double-orifice mitral valve is a high risk factor for operative death in total endocardial cushion defect and the longer term results of partial endocardial cushion defect combined with double-orifice mitral valve is not satisfactory.

Congenital Double-Orifice Mitral Valve with Mitral Valve Prolapse and Severe Mitral Regurgitation

A 27 year-old female presented with dyspnea on exertion, and was diagnosed using transthoracic and transesophageal echocardiography as congenital double-orifice mitral valve and mitral valve prolapse resulting in severe mitral regurgitation. This finding was confirmed by open heart surgery, and her mitral valve was successfully repaired with lateral commissural repair and ring annuloplasty using da Vinci system. We report this case with review of literature.

Real-time Three-dimensional Echocardiography in Assessment of Congenital Double Orifice Mitral Valve / 华中科技大学学报（医学）（英德文版）

The application of real-time three-dimensional echocardiography (RT 3DE) in the diagnosis of double orifice mitral valve (DOMV) was explored. Five cases of DOMV were examined by using 2-dimensional echocardiography (2DE) and RT 3DE. The spatial morphology of malformed mitral valve and its change in hemodynamics were observed. DOMV associated with partial atrioventricular septal defect was found in 3 cases (in which 2 cases had cleft mitral valve) and isolated DOMV in 2 cases; and moderate to severe mitral regurgitation was detected in 3 cases, and mild mitral regurgitation in 1, and no regurgitation in 1 case; 1 case had complicated rhumatic heart disease.Three cases were preoperatively discovered by 2DE, while 2 missed (1 case was discovered postoperatively). Four cases were diagnosed by RT 3DE preoperatively, and 1 case was diagnosed postoperatively (not examined by RT 3DE preoperatively). It was suggested that RT 3DE is a reliable technique in the diagnosis of DOMV; it permitted comprehensive and noninvasive assessment of mitral valve and may supplement 2D TTE in the assessment of DOMV.

Congenital Double-Orifice Mitral Valve with Mitral Regurgitation due to Flail Leaflet in an Elderly Patient

We report here on a case of double-orifice mitral valve with mitral regurgitation in a 75-year-old female who had complaints of mild dyspnea. Transthoracic and transesophageal echocardiography showed two orifices that were supplied by their own chordae from a different papillary muscle. Color Doppler echocardiography revealed moderate to severe mitral regurgitation due to the flail posterior leaflet of the anterolateral orifice. Except for the persistent left superior vena cava, no other congenital anomaly was demonstrated. The patient became asymptomatic with the administration of angiotensin-converting enzyme inhibitor and diuretics, and she has been scheduled for long term follow-up.

A Case of Isolated Congenital Double-Orifice Mitral Valve

Double-orifice mitral valve is a relatively rare congenital abnormality, usually discovered at autopsy or surgery. In most cases, the double-orifice mitral valve causes no hemodynamic effects, sometimes it is regurgitant, and rarely is stenotic. Appreciation of this echocardiographic abnormality is important because double orifice mitral valve is often associated with other congenital anomalies and this echocardiographic findings may be confused with other cardiac abnormalities. The authors report a case of isolated congenital double-orifice mitral valve in a 42-year-old woman. Data from the literature are reviewed and the echocardiographic images of the malformation are described.

Two Cases of Double-Orifice Mitral Valve Detected by Echocardiography

Double-orifice mitral valve is a rare congenital anomaly. Most cases of double-orifice mitral valve are hemodynamically normal and remain symptomless, so that it is usually discovered incidentally in autopsy or during surgical correction of a cardiovascular abnormality. Recently, however, it is increasingly recognized as such, since the echocardiography has gained wide acceptance as a non-invasive diagnostic tool by the M-mode, two-dimensional and color Doppler echocardiogram. Two separate mitral valve apparatuses can be used on the M-mode echocardiogram. In the two-dimensional echocardiography, the parasternal and subcostal short-axis views can show two separate glass-like orifices in the left ventricle, and the parasternal long-axis view as well as the apical four-chamber view can show the anomaly. And the color Doppler echocardiogram can visualize two mosaic-pattern flows between the left atrium and ventricle. We present herewith two cases of double-orifice mitral valve, as diagnosed by means of echocardiography. The first case was an isolated one with mitral stenosis, showing two parachute mitral valves. The second was associated with perimembranous ventricular septal defect, and showed the accessory mitral valve directly attached to the ventricular septum, with the chordae crossing the ventricular outflow tract.

Tricuspid atresia associated with double orifice mitral valve and coronary sinus septal defect

No abstract available.