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Year : 2017  |  Volume : 5  |  Issue : 3  |  Page : 133-135

Double orifice mitral valve

Department of Cardiology, Fortis Escorts Hospital, Jaipur, Rajasthan, India

Date of Web Publication12-Sep-2017

Correspondence Address:
S Khan
Department of Cardiology, Fortis Escorts Hospital, Jaipur - 302 017, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/heartindia.heartindia_17_17

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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. Subvalvular structures, especially the tensor apparatus, invariably shows various degrees of abnormalities. DOMV may usually allow normal blood flow between the left atrium and left ventricle but can sometimes substantially obstruct mitral valve inflow or produce mitral valve regurgitation. Recognition of DOMV and awareness of the anatomic variations are important to achieve good therapeutic results. Treatment is only necessary if significant mitral stenosis or mitral regurgitation is present. Here, we present a rare case of DOMV with ventricular septal defect which was detected by two-dimensional echocardiography.

Keywords: Congenital, double orifice mitral valve, heart defects, ventriclular septal defects

How to cite this article:
Khan S, Soni V, Khatri S, Roy S. Double orifice mitral valve. Heart India 2017;5:133-5

How to cite this URL:
Khan S, Soni V, Khatri S, Roy S. Double orifice mitral valve. Heart India [serial online] 2017 [cited 2020 Jun 4];5:133-5. Available from: http://www.heartindia.net/text.asp?2017/5/3/133/214423

  Introduction Top

Double orifice mitral valve (DOMV) is a rare congenital malformation which was first reported by Greenfield in 1876. It is often associated with other cardiac anomalies.[1] It usually does not lead to any significant hemodynamic effects except a regurgitant and rarely stenotic mitral valve.

  Case Report Top

A 4-month-old male child presented with a history of rapid breathing and failure to gain weight.

On physical examination

  • HR: 115/min
  • CVS: Normal S1 with split S2; A2 = P2, ejection systolic murmur (Grade 3/6) audible in third, fourth, and fifth intercostal space, in left parasternal area
  • Chest: Clear B/L.

On evaluation

  • Electrocardiogram-normal, chest X-ray: Mild cardiomegaly and increased lung vasculature
  • Transthoracic echocardiography (2D and 3D).[2],[3]

Dilated left atrium and left ventricle, mitral valve was divided into two separate valve orifices by a fibrous bridge.

During diastole (in four chamber view), the anterior mitral leaflet appeared like a “V” due to an abnormal bridge of fibrous tissue connecting the two leaflets.

Color Doppler flow imaging of the mitral valve showed two separate envelopes of antegrade flow into the left ventricle through the double orifice during diastole. No mitral or aortic regurgitation was documented by Color Doppler flow imaging. The left ventricular ejection fraction was within normal limits. There was a moderate size perimembranous ventricular septal defect (VSD) with left to right shunt.

In view of patient's age and the cardiac lesion, i.e., DOMV associated with small VSD without mitral valve obstruction, patient was advised conservative management with regular follow-up.
Figure 1: DOMV 3D image reconstruction

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Figure 2: DOMV PSAX view TTE

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Figure 3: VSD PlAX view TTE

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Figure 4: DOMV 4C view TTE

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  Discussion Top

The normal mitral valve consists of a central orifice located between a sail-like anterior leaflet and a C-shaped posterior leaflet. The anterior mitral leaflet occupies roughly one-third of the annular circumference, and the posterior leaflet occupies roughly the remaining two-thirds of the annular circumference. In a DOMV, however, abnormal tissue divides the mitral orifice into two parts. Mitral orifice are unequal in size, with the smaller orifice directed toward the anterolateral commissure (41%) or the posteromedial commissure (44%).

The mitral valve can function reasonably well in about 50% of patients with DOMV.[3] In the other 50%, it can cause clinically significant mitral stenosis or mitral regurgitation. The commonly associated lesion is atrioventricular (AV) septal defect, VSD, coarctation of aorta, interrupted aortic arch, patent ductus arteriosus, primum atrial septal defect, tetralogy of fallot, and abstein anomaly.

Rosenberg et al. reported 25% of patients with DOMV have partial persistent AV canal and about 5% of patients with partial persistent AV canal have DOMV. Das et al. found that various abnormalities along with DOMV were left-sided obstructive lesions, VSD; anomalies of the tricuspid valve. However, in our case there was only a VSD as an associated anomaly.

The classification based on echocardiographic imaging was proposed by Trowitsch et al. which divided DOMV into three different types:[4]

  1. Hole type (accessory orifice surrounded by leaflet tissue that may have a chordal ring)
  2. Complete bridging (fibrous bridge in the plane of mitral valve sails, dividing mitral valve opening into two parts that may be equal or unequal)
  3. In complete bridging (small strand of fibrous tissue connects only the tips of anterior and posterior leaflets).

Our patient falls into the complete type of DOMV as per Trowitsch classification. Rosenberg et al. reported that the true developmental basis of duplication of mitral valve is unknown. DOMV might be the result of fetal endocarditis or may be purely developmental anomaly.[5]

Another study proposed that the double orifice mitral valve was the result of an early arrest of development and that the accessory orifice represents retention of the left portion of the common AV canal with subsequent reduction of mitral ostium and alignment with it.

Management depends on type and severity of mitral valve dysfunction. Isolated DOMV causing neither obstruction nor regurgitation needs no active intervention. Surgical intervention is necessary when stenosis or incompetence is severe or if repair of an associated cardiac lesion is needed. Long-term follow-up is necessary to detect subsequent abnormal hemodynamics or complications. More recently, two-dimensional echocardiography has allowed a noninvasive and more frequent detection of this anomaly.

  Conclusion Top

This case demonstrates the necessity of careful imaging of the mitral valve apparatus in patients with VSDs.[6]

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Conflicts of interest

There are no conflicts of interest.

  References Top

Taksande A, Goutami V, Thomas E. Double orifice mitral valve associated with Ventricular Septal Defect in an infant: Case Report. Images Paediatr Cardiol 2011;13:6-9.  Back to cited text no. 1
Abdullah M, Pearce K, Palmer N, Chenzbraun A. Double orifice mitral valve with dysplastic tricuspid valve and intact interatrial septum: A three-dimensional echocardiographic study. Eur J Echocardiogr 2008;9:598-9.  Back to cited text no. 2
Anwar AM, McGhie JS, Meijboom FJ, Ten Cate FJ. Double orifice mitral valve by real-time three-dimensional echocardiography. Eur J Echocardiogr 2008;9:731-2.  Back to cited text no. 3
Trowitzsch E, Bano-Rodrigo A, Burger BM, Colan SD, Sanders SP. Two-dimensional echocardiographic findings in double orifice mitral valve. J Am Coll Cardiol 1985;6:383-7.  Back to cited text no. 4
Baño-Rodrigo A, Van Praagh S, Trowitzsch E, Van Praagh R. Double-orifice mitral valve: A study of 27 postmortem cases with developmental, diagnostic and surgical considerations. Am J Cardiol 1988;61:152-60.  Back to cited text no. 5
Linka AZ, Fatio R, Attenhofer Jost C. Images in cardiology: Double orifice mitral valve. Heart 2000;84:244.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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