Heart India

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

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


Shamsher Singh, Ratna Pandey, Alok Kumar Singh, Ajay Kumar Pandey 
 Department of Cardiology, Heritage Hospital, Varanasi, Uttar Pradesh, India

Correspondence Address:
Ajay Kumar Pandey
Department of Cardiology, Heritage Hospital, Varanasi 221 005, Uttar Pradesh
India




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;1:20-21


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 2021 Jun 18 ];1:20-21
Available from: https://www.heartindia.net/text.asp?2013/1/1/20/113600


Full Text

 Case Report



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}{Figure 2}{Figure 3}

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

1Greenfield WS. Double mitral valve. Trans Pathol Soc 1876;27:128-9.
2van 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.