ABSTRACT

A double orifice mitral valve (DOMV) represents a rare congenital malformation characterized by two valve orifices with two separate subvalvular apparatus. We present an interesting case of an asymptomatic 1 year and 8 months old male child with DOMV who successfully underwent surgery.

Introduction

A double orifice mitral valve (DOMV) is an uncommon anomaly that was first described by Greenfield in 1876. DOMV represents a rare congenital malformation characterized by two valve orifices with two separate subvalvular apparatus.

Case Report

We report a case of an asymptomatic 1 year and 8 months old male child, referred to our department by a pediatric cardiologist. His echocardiography finding suggestive of partial atrioventricular (AV) canal defect with DOMV with the small mitral annulus [Figure 1]. Tricuspid annulus was 14 mm whereas mitral annulus was 9 mm.

During surgery, DOMV anatomy was assessed [Figure 2], the valve area was adequate for his body surface area without any regurgitation. 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 follow-up where he can develop regurgitation. The patient is on regular follow-up without any mitral valve regurgitation or stenosis for 6 months, and he is doing well.

Discussion

A DOMV is an uncommon anomaly that was first described by Greenfield in 1876.^[1] DOMV represents a rare congenital malformation. Characterized by two valve orifices with two separate subvalvular apparatus.^[2],[3] The natural history of DOMV with intact AV septum is not known.

This case reveals the necessity of careful imaging of the mitral valve apparatus, not only in patients with AV septal defects but also in patients with congenital left obstructive heart disease.

It is most often associated with AV septal defects (it has been found in as many as 4.9%–17.9% of cases in necropsy studies),^[4] 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 hemodynamic 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. However, these patients need regular follow-up to know any change in morphology and function of the left AV valve in the near future.

Declaration of patient consent

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Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.