European Journal of Echocardiography 2006 7(6):463-464; doi:10.1016/j.euje.2005.08.007
Copyright © 2005, The European Society of Cardiology
Double orifice mitral valve; a coincidental finding*
Iris C.D. Westendorpa,*,
H.A.C.M. de Bruin-Bonb and
Jana Hrudovab
aOnze Lieve Vrouwe Gasthuis (OLVG), Department of Cardiology, P.O. Box 95500, 1091 HA, Amsterdam, The Netherlands
bAcademic Medical Center (AMC), Department of Cardiology, Amsterdam, The Netherlands
Received 29 July 2005; accepted after revision 30 August 2005.
* Corresponding author. Tel.: +31 20 5999111; fax: +31 20 5993779. iwestendorp{at}cs.com
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Abstract
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A double orifice mitral valve (DOMV) represents a rare congenital
malformation characterised by two valve orifices with two separate
subvalvular apparatus. This case demonstrates the necessity
of careful imaging of the mitral valve apparatus, not only in
patients with atrioventricular septal defects, but also in patients
with congenital left obstructive heart disease.
Keywords: Mitral valve; Double orifice mitral valve; Congenital heart disease; Echocardiography
* No financial support was received for this study. There are no conflicts of interest to report. 
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Case description
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A double orifice mitral valve (DOMV) represents a rare congenital
malformation characterised by two valve orifices with two separate
subvalvular apparatus (
Figs. 1–3
).
1,2 It is most often
associated with atrioventricular septal defects, but may also
be present together with other congenital heart defects such
as left-sided obstructive lesions, ventricular septal defects
or cyanotic lesions. Rarely, patients with isolated DOMV are
reported. However, even in the modern era of echocardiography
DOMV often remains unrecognized. The haemodynamic impact of
DOMV varies from a normally functioning valve to (less frequent)
significant mitral regurgitation (with or without cleft) or
stenosis. Surgical correction of DOMV is indicated in a minority
of patients undergoing repair of associated lesions. The natural
history of DOMV with intact atrioventricular septum is not known.
We report a case of an asymptomatic 24-year-old man, referred
to our department by his paediatric cardiologist. At the age
of 1 he underwent correction for an aortic coarctation. Since
then he has been reviewed regularly in our outpatient clinic
for a bicuspid aortic valve with moderate to severe aortic stenosis
and a moderately dilated ascending aorta, which will require
future surgery. Routine echocardiography showed a previously
non-recognized DOMV, with slight mitral regurgitation through
both orifices. At this moment, recognition of DOMV has no impact
on the patient's management. It might, however, be of importance
at the time of a further operation.
This case demonstrates the necessity of careful imaging of the mitral valve apparatus, not only in patients with atrioventricular septal defects, but also in patients with congenital left obstructive heart disease.
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Notes
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* No financial support was received for this study. There are
no conflicts of interest to report.
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References
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- Zalzstein E., Hamilton R., Zucker N., Levitas A., Gross G.J. Presentation, natural history, and outcome in children and adolescents with double orifice mitral valve. Am J Cardiol (2004) 93(8):1067–1069.[CrossRef][Web of Science][Medline]
- Das B.B., Pauliks L.B., Knudson O.A., Kirby S., Chan K.-C., Valdez-Cruz L., et al. Double-orifice mitral valve with intact atrioventricular septum: an echocardiographic study with anatomic and functional considerations. J Am Soc Echocardiogr (2005) 18:231–236.[CrossRef][Web of Science][Medline]
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