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European Journal of Echocardiography Advance Access originally published online on March 20, 2008
European Journal of Echocardiography 2008 9(4):598-599; doi:10.1093/ejechocard/jen128
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2008. For permissions please email: journals.permissions@oxfordjournals.org

Double orifice mitral valve with dysplastic tricuspid valve and intact interatrial septum: a three-dimensional echocardiographic study

Mohammad Abdullah, Keith Pearce, Nick Palmer and Adrian Chenzbraun*

The Cardiothoracic Centre, Liverpool NHS Trust, Royal Liverpool University Hospital, Prescott Street, Liverpool, UK

Received 22 November 2007; accepted after revision 24 February 2008; online publish-ahead-of-print 20 March 2008.

* Corresponding author. Tel: +44 0151 7063488. E-mail address: a.chenzbraun{at}btinternet.com


   Abstract
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 Abstract
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Double orifice mitral valve is a rare congenital heart disease. We present a case in which 3D echocardiography helped clarify the underlying anatomy. The need exists for more extensive use of this technique to allow for surgical and pathological confirmation.

Keywords: 3D echocardiography; Double orifice mitral valve; Congenital heart disease

A 26-year-old male patient with a known diagnosis of congenital heart disease was referred to routine cardiological assessment. The patient was asymptomatic and no details were available about his cardiac condition.

In parasternal long axis view, echocardiography showed what seemed to be a rheumatic valve with mild mitral stenosis and no regurgitation. Parasternal short axis view, however, revealed a double orifice mitral valve (DOMV) with the larger orifice situated laterally (Figure 1). Tricuspid valve was dysplastic with tethered posterior leaflet (Figure 2), borderline apical displacement of the septal tricuspid leaflet of 1 cm, and severe tricuspid regurgitation. There was no other cardiac abnormality and a contrast study was negative for shunt. Real-time 3D scanning (Vivid 7, General Electric Medical Systems, Milwaukee, Wisc, USA) provided good visualization of the mitral valve when looked at from the apex in both parasternal (see Supplementary data online, Video 1) and apical views (see Supplementary data online, Video 2) showing a full bridge at leaflets edges level (see Supplementary data online, Video 1) but became partial only when the cutting plane was translated towards the annulus (see Supplementary data online, Video 3). Three-dimensional interrogation of the tricuspid valve confirmed the tethering of the posterior leaflet and allowed excellent morphological assessment of the regurgitant orifice with lack of leaflets coaptation at the posterior aspect of the valve (see Supplementary data online, Video 4).


Figure 1
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  		Figure 1 Diastolic frame of the mitral valve in parasternal long axis view, showing two circular mitral orifices of unequal size.

 


Figure 2
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  		Figure 2 Systolic frame of the tricuspid valve in a long axis view of the right ventricle inflow. The posterior tricuspid leaflet is completely restricted and tethered whereas the anterior leaflet is distorted and partially restricted.

 
Double orifice mitral valve is a rare congenital malformation. It has been associated with various other congenital defects, mainly left heart obstructive lesions and AV canal,1–3 and the clinical picture varies from symptomatic to unexpected necropsy findings. The anatomical classification relies on identifying the extent of the fibrous ridge between the leaflets.4 In 2D echocardiography, this requires careful scanning from apex to base in parasternal short axis view. This provides a limited perspective and is not technically satisfactory in all patients. We describe an asymptomatic case of DOMV with associated dysplastic tricuspid valve. Combined tri-dimensional scanning in both parasternal and apical views identified a fibrous ridge at leaflets edge level only, suggesting an incomplete ridge-type DOMV. The added diagnostic value of 3D echo in this case was noteworthy and needs to be established in additional cases with surgical corroboration.


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Supplementary data are available at European Journal of Echocardiography online.


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  1. Bano-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.[CrossRef][Web of Science][Medline]
  2. Das BB, Pauliks LB, Knudson OA, Kirby S, Chan KC, Valdes-Cruz L, et al. Double-orifice mitral valve with intact atrioventricular septum: an echocardiographic study with anatomic and functional considerations. J Am Soc Echocardiography (2005) 18:231–6.[CrossRef][Web of Science][Medline]
  3. Yamaguchi M, Tachibana H, Hosokawa Y, Ohoshi H, Oshima Y, Obo H. Ebstein's anomaly and partial atrioventricular canal associated with double orifice mitral valve. J Cardiovasc Surg (1989) 30:790–2.[Medline]
  4. Trowitzsch E, Bano-Rodrigo A, Burger BM. Two-dimensional echocardiographic findings in double-orifice mitral valve. J Am Coll Cardiol (1985) 6:383–7.[Abstract]

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