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Year : 2013  |  Volume : 1  |  Issue : 1  |  Page : 20-21

A Rare Case of Double Orifice Mitral Valve with Multiple Muscular Ventricular Septal Defect


Department of Cardiology, Heritage Hospital, Varanasi, Uttar Pradesh, India

Date of Web Publication17-Jun-2013

Correspondence Address:
Ajay Kumar Pandey
Department of Cardiology, Heritage Hospital, Varanasi 221 005, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2321-449x.113600

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How to cite this article:
Singh S, Pandey R, Singh AK, Pandey AK. A Rare Case of Double Orifice Mitral Valve with Multiple Muscular Ventricular Septal Defect. Heart India 2013;1:20-1

How to cite this URL:
Singh S, Pandey R, Singh AK, Pandey AK. A Rare Case of Double Orifice Mitral Valve with Multiple Muscular Ventricular Septal Defect. Heart India [serial online] 2013 [cited 2020 Jul 13];1:20-1. Available from: http://www.heartindia.net/text.asp?2013/1/1/20/113600


  Case Report Top


A 25-year-old boy with a history of progressive breathlessness for 2 year referred to Heritage Hospital echocardiography lab on 16 th March 2012 for echocardiography examinations by local physician. Physical examination was unremarkable except for grade 4 pansystolic murmur in the left parasternal area and spo2 by pulse oxymeter was 95%. His 2D Echocardiography (ECHO) shows multiple muscular Ventricular Septal Defect (VSD) with bidirectional flow (predominant left to right shunt) and double orifice mitral valve (DOMV) [Figure 1], [Figure 2], [Figure 3]. Mitral valve is functionally normal without stenosis and regurgitation and there was no tricuspid regurgitation jet visible. He was advised for cardiac catheterization, but patient refused for the same. He was on frusemide 20 mg twice daily. We are reporting this case because of rare combination of muscular VSD with DOMV.
Figure 1: Parasternal short axis view showing double orifice mitral valve

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Figure 2: An apical four chamber view showing double orifice mitral valve

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Figure 3: An apical chamber view showing multiple muscular ventricular septal defects

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DOMV is a rarely reported anomaly was first described by Greenfield in 1876. [1] This anomaly is characterized by a mitral valve with a single fibrous annulus with two orifices that open into the left ventricle. In most cases (85%), a larger orifice is accompanied by a small eccentric accessory orifice known as eccentric variety of DOMV, and second uncommon type of DOMV is concentric DOMV (15%) in which both mitral orifices are equal. Embryologically the lesion results from persistence of the left part of the common atrio-ventricular canal and abnormal leaflet fusion. In about 50% of DOMV cases, valvular function is normal, others present with stenosis or regurgitation. [2] Definitive management of most of the DOMV patients is surgical replacement of mitral valve.

 
  References Top

1.Greenfield WS. Double mitral valve. Trans Pathol Soc 1876;27:128-9.  Back to cited text no. 1
    
2.van Buuren F, Faber L, Bogunovic N. Double orifice mitral valve with normal function: An echocardiography and MRI study of a rare finding. Eur Heart J 2011;32:137.  Back to cited text no. 2
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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