European Journal of Echocardiography Advance Access published online on May 1, 2008
European Journal of Echocardiography, doi:10.1093/ejechocard/jen149
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Double orifice mitral valve by real-time three-dimensional echocardiography
1 Department of Cardiology, Thoraxcenter, Room Ba 302, Erasmus MC, Dr. Molewaterplein 40, 3015 GD Rotterdam, The Netherlands
2 Department of Cardiology, Al-Husein University Hospital, Al-Azhar University, Cairo, Egypt
Received 25 December 2007; accepted after revision 21 March 2008.
* Corresponding author. Tel: +31 10 4633986; fax: +31 10 4635498. E-mail address: f.j.tencate{at}erasmusmc.nl
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Abstract |
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Double orifice mitral valve (DOMV) is a rare congenital malformation described as division of mitral orifice into two anatomically distinct orifices separated by an accessory bridge of fibrous tissue. In 85% of cases, both orifices are unequal in size. It is usually associated with other congenital defects such as atrioventricular septal defect and complex congenital heart disease. Most of cases could be diagnosed by two-dimensional echocardiography (2DE). The real-time three-dimensional echocardiography (RT3DE) helped in more detailed structure and function. Presented here RT3DE used for orientation of DOMV that allowed detailed and comprehensive assessment incremental to that obtained by 2DE.
Keywords: Double orifice mitral valve; Real-time three-dimensional echocardiography; Congenital heart disease
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Introduction |
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Double orifice mitral valve (DOMV) was described for the first time by Greenfield in 1876. Since that time, more than 200 cases have been reported.1 The resulting lesion consists of two anatomically distinct orifices separated by an accessory fibrous tissue. The most common type (account for 85%) is eccentric or hole type that is characterized by a small accessory orifice situated at one of commissures. Other less common types are central type (account for 15%) and duplicate mitral valves.2 The embryologic theories explaining its origin include abnormal leaflets fusion and persistence of left part of the common atrio-ventricular canal. DOMV can occur as an isolated anomaly but usually, it is associated with other congenital anomalies as endocardial cushion defect, bicuspid aortic valve, aortic coarctation, interrupted aortic arch, patent ductus arteriosus, and complex congenital heart disease.3 The mitral valve has reasonable function in about 50% of patients and significant stenosis or regurgitation is clinically presented in the other 50%. By trans-thoracic and trans-esophageal two-dimensional echocardiography (2DE), most of cases could be diagnosed.4,5 We described a case of double orifice mitral valve fully assessed by real-time three-dimensional echocardiography (RT3DE).
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Case study |
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A 19-year-old woman was referred to our echocardiography lab for evaluation of systolic heart murmur. She was in good physical condition, with skeletal deformity (pectus excavatum), a 72 bpm regular and equal pulse, and blood pressure 140/70 mmHg. No peripheral oedema and no enlarged liver were found. Cardiac examination revealed a thrill and by auscultation a loud apical pansystolic murmur (grades III–IV) and ejection systolic murmur was heard in the aortic area. ECG showed normal sinus rhythm with evidence of right ventricular hypertrophy. Chest X-ray demonstrated mild cardiomegaly. Transthoracic 2DE was done with Philips Sonos 7500 ultrasound machine showed ostium primum atrial septal defect (ASD), dilated left atrium (44 mm), grade III mitral regurgitation, and aortic coarctation. Mitral valve was domed on parasternal long-axis view. In short-axis view, it was seen incompletely divided by fibrous tissue into two orifices but no further details could be obtained. RT3DE was done using same machine and 4x matrix transducer confirmed the DOMV diagnosis by enface view from the ventricular surface (Figure 1). A central bridge of fibrous or abnormal leaflet tissue connects the two leaflets of the mitral valve, dividing the orifice into two unequal orifices (medial and lateral) was seen. The medial one was the larger (Figure 2). The papillary muscles were normal, with chordate surrounding each orifice inserting into two papillary muscles. The patient was then accepted for surgical closure of ASD, correction of coarctation, and MV replacement. The surgical findings confirmed the RT3DE description of the mitral valve.
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Discussion |
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DOMV is an uncommon congenital cardiac anomaly characterized by a mitral valve with a single fibrous annulus and two orifices. The clinical presentation and management depend on the type and severity of mitral valve dysfunction and also the associated congenital defects.2 In asymptomatic patient, DOMV can be an incidental finding during echocardiographic examination. Our patient was asymptomatic and referred to echocardiography to evaluate systolic murmur during routine examination. Chest X-ray and resting ECG were not conclusive.
The diagnosis of DOMV depends mainly on 2DE through assessment of mitral valve morphology and function. However, information obtained by 2DE may be incomplete especially with poor image quailty.6 In our patient, 2DE helped in the diagnosis of DOMV and detected the severity of mitral regurgitation beside the other associated anomalies (ostium primum ASD and aortic coarctation). RT3DE adds more information by recognition of the anatomical character of valve orifices (size and spatial relationship) and function. In accordance with Lu et al.7 who suggested that RT3DE permit a reliable and comprehensive assessment of DOMV (four cases preoperatively and one case postoperatively), the RT3DE data were complementary to 2DE and was helpful during surgery.
All patients with significant mitral stenosis or regurgitation require medical therapy to relieve pulmonary congestion and to stabilize him before intervention (transcatheter or surgical). Surgical intervention is necessary when stenosis or incompetence is severe or if repair of an associated cardiac lesion is needed. Surgical intervention was decided to our patient due to presence of grade III mitral regurgitation and associated aortic coarctation and ASD. The type of operation depends on the detailed anatomical and functional description of the mitral valve abnormality. Mitral valve repair and reconstruction procedures are preferred unless the valve is markedly abnormal where the replacement is necessary.3
The practical message from this case report is that in patient with DOMV, application of RT3DE allows comprehensive non-invasive assessment of the anatomical details and provides incremental information to that obtained by 2DE.8 The second message is that DOMV can be masked during evaluation of tetralogy of Fallot and large left to right atrial shunt and thus need full assessment.
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References |
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- Purnode P, Rombaut E, Gerard M, Alkhori M, Marchandise B. Double orifice mitral valve with flail leaflet: a transoesophageal echocardiographic examination. Eur J Echocardiogr (2000) 1:144–6.
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