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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.
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@#Objective To investigate the early and mid-term clinical outcomes of the modified cone reconstruction in the treatment of Ebstein’s anomaly (EA). Methods Clinical data of 18 consecutive patients with EA in our hospital between May 2008 and August 2015 were retrospectively analyzed. All patients were diagnosed by echocardiography. There were 8 males and 10 females with an average age of 20.3 years ranging from 5 to 41 years. According to New York Heart Association classification, 12 patients were classified into grade Ⅱ and 6 grade Ⅲ. One patient had acute arterial embolism and amputation of left lower extremity caused by paradoxical embolism of combined secundum atrial septal defect, and another one was combined with double-orifice technique due to postoperative poor closure of tricuspid valve. The modified cone reconstruction was used to correct the EA, to make leaflets coapted well and form central blood flow. For those patients whose anterior leaflet developed poor and smaller, valve leaflet was widened by using autologous pericardial. For all patients, tricuspid annulus was reinforced by autologous pericardial. Results Two patients suffered arrhythmia, and returned to normal after medication. The rest patients recovered well without death. Echocardiography found 1 patient with moderate regurgitation and the rest of patients’ leaflets coapted well and had no tricuspid stenosis. They were followed up 9 to 38 months postoperatively, and cardiac function of gradeⅠin 14 patients and gradeⅡin 4 patients. Conclusion The early and mid-term clinical outcomes of the modified cone reconstruction in the treatment of EA are affirmative which can make leaflets coapt completely and have a strong anti-regurgitation ability, reducing the incidence of re-operation, valve replacement and postoperative mortality.
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No abstract available.
Subject(s)
Echocardiography , Echocardiography, Three-Dimensional , Tricuspid ValveABSTRACT
Mitral valve having a double orifice opening into the left ventricle is an uncommon anomaly, In this case study we describe about a 25 years old female who came to cardiology outpatient block with exertional dysnea , the abnormality was suspected by a murmur ,further investigation Colour Doppler echocardiography showed congenital double-orifice mitral valve. This finding was confirmed by open heart surgery, and her mitral valve was successfully repaired.
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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.
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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.
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We describe two cases of a 9-day-old male and a 5-year-10-month-old female who had congestive heart failure due to severe mitral regurgitation. Preoperative echocardiogram revealed severe mitral regurgitation due to dysplasia of the leaflet, chordae and papillary muscle. Because the leaflet area was not enough for the valve orifice, we performed double orifice repair by suturing the free edge to the corresponding edge of the opposite leaflet. The mitral regurgitation was found to be significantly reduced on postoperative echocardiogram. Double orifice repair is simple and can be performed rapidly. However, although this technique has been performed in adults, it has not been commonly performed in infants. Our results suggest that this procedure can be useful for the congenital mitral regurgitation. Long-term follow-up is needed to confirm the result.
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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.
Subject(s)
Female , Humans , Dyspnea , Echocardiography, Transesophageal , Mitral Valve , Mitral Valve Insufficiency , Mitral Valve Prolapse , Thoracic SurgeryABSTRACT
A 33-year-old man presented with respiratory distress and lower leg edema in April 2006. Atrial septal defect (ASD), complicated with moderate mitral regurgitation (MR), advanced tricuspid regurgitation (TR) and pulmonary hypertension (95/32mmHg), was diagnosed. Qp/Qs was 6.3 and L-R shunt ratio was 84.4%. An “edge-to-edge” mitral valve plasty for MR complication as well as closure of the septal defect and tricuspid annuloplasty was performed, and a good result was obtained. It is known that ASD has a tendency to be accompanied by MR, and the strategy for treatment course for MR is debatable. The mitral lesions of MR complicating ASD are often seen in the posteromedial side of the anterior mitral leaflet, and usually many of the tendinous cords and valve leaflets are in the normal range in length. There have been reports on the mid-term results of edge-to-edge repair of mitral regurgitation due to degenerative lesions but the mid- and long-term results for MR complicating ASD, such as this case are unknown. We need to carefully observe the time course of this case.
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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.
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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.
Subject(s)
Humans , Female , Aged , Papillary Muscles/abnormalities , Mitral Valve Insufficiency/etiology , Mitral Valve/abnormalities , Echocardiography, Doppler, Color , Chordae Tendineae/abnormalitiesABSTRACT
Objective: To evaluate the clinical effect of the double-orifice technique in the treatment of the anterior leaflet prolapse of mitral valve. Methods: 35 patients with severe anterior leaflet prolapse of mitral valve underwent valve repair using the double-orifice technique. The condition of valve was assessed with echocardiography preoperatively, intraoperatively, and postoperatively. Results: The mean postoperative valve area was (4 1?1 8) cm 2 against the preoperative valve area of (8 3?1 6)cm 2. Mild regurgitation was found in 2 patients after operation. There were no hospital deaths. At the latest follow-up, the heart function was improved significantly in all patients (NYHA class I). Echo-Doppler assessment of valve showed stable valve function in all patients. There were no late stenosis of valve and no late death. Conclusion: The results of this study show that the double-orifice technique is a safe and effective method for treatment of anterior leaflet of mitral valve with low mortality and morbidity, and excellent early and mid-term results.
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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.
Subject(s)
Adult , Female , Humans , Autopsy , Congenital Abnormalities , Echocardiography , Hemodynamics , Mitral ValveABSTRACT
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.