J Cardiovasc Ultrasound.  2008 Sep;16(3):87-89. 10.4250/jcu.2008.16.3.87.

Congenital Double-Orifice Mitral Valve with Mitral Valve Prolapse and Severe Mitral Regurgitation

• Lee JW
• Song JM
• Park JP
• Kang DH
• Song JK

• KMID: 1486584
• DOI: http://doi.org/10.4250/jcu.2008.16.3.87

Abstract

A 27 year-old female presented with dyspnea on exertion, and was diagnosed using transthoracic and transesophageal echocardiography as congenital double-orifice mitral valve and mitral valve prolapse resulting in severe mitral regurgitation. This finding was confirmed by open heart surgery, and her mitral valve was successfully repaired with lateral commissural repair and ring annuloplasty using da Vinci system. We report this case with review of literature.

Keyword

Double-orifice mitral valve; Mitral valve prolapse; Mitral regurgitation

MeSH Terms

Dyspnea
Echocardiography, Transesophageal
Female
Humans
Mitral Valve
Mitral Valve Insufficiency
Mitral Valve Prolapse
Thoracic Surgery

Figure

• 

  Fig. 1 Transthoracic echocardiography shows that (A) mitral valve is divided into 2 separate valve orifices (anterolateral & posteromedial, arrows) by a fibrous bridge on the parasternal short axis view, and (B) center part of anterior mitral leaflet (arrow), fibrous bridge does not move toward apex at diastole while the medial and lateral parts do on the apical 2-chamber view.

• 

  Fig. 2 Transesophageal echocardiography shows that (A) center part of anterior mitral leaflet (arrow), fibrous bridge not moving toward apex during diastole and (B) the prolapsed lateral scallop of the posterior mitral valve leaflet (P1 segment, arrow) into the left atrium during systole. The color Doppler image shows (C) 2 separate mitral inflows through 2 separate orifices, and (D) severe eccentric mitral regurgitation jet toward medial side of the left atrium.

• 

  Fig. 3 Intraoperative view of the mitral valve from the left atrial side shows 2 different mitral orifices (arrows) with equal size and central fibrous bridge. Arrowhead indicates prolapsed segment.

Reference

1. Greenfield WS. Double mitral valve. Trans Path Soc London. 1876. 27:128–129.

2. Rosenberg J, Roberts WC. Double-orifice mitral valve. Arch Pat. 1968. 86:77–80.

3. Bano-Rodrigo A, van Praagh S, Trowitzsch E, van Praahg R. Double-orifice mitral valve: a study of 27 postmortem cases with developmental, diagnostic and surgical considerations. Am J Cardiol. 1998. 61:152–160.

4. Zalzstein E, Hamilton R, Zucker N, Levitas A, Gross GJ. Presentation, natural history, and outcome in children and adolescents with double orifice mitral valve. Am J Cardiol. 2004. 93:1067–1069.

5. Trowitzsch E, Bano-Rodrigo A, Burger BM, Colan SD, Sanders SP. Two-dimensional echocardiographic findings in double orifice mitral valve. J Am Coll Cardiol. 1985. 6:383–387.

6. Das BB, Pauliks LB, Knudson OA, Kirby S, Chan KC, Valdes-Cruz L, Cayre RO. Double-orifice mitral valve with intact atrioventricular septum: an echocardiographic study with anatomic and functional considerations. J Am Soc Echocardiogr. 2005. 18:231–236.

7. Matsuwaka R, Sakakivara T, Mitsuno M, Yagura A, Yoshikawa M, Ishikura F. Valve repair for mitral regurgitation associated with isolated double-orifice mitral valve. J Thorac Cardiovasc Surg. 1996. 112:1666–1667.

8. Tomita Y, Yasui H, Tominaga R. Mitral valve repair for isolated double-orifice mitral valve with torn chordae. Ann Thorac Surg. 1997. 64:1831–1834.

9. Shiran A, Lewis BS. Acquired partially flail leaflet causing severe mitral regurgitation in a congenital double-orifice mitral valve. J Am Soc Echocardiogr. 2004. 17:478–479.

10. Congiu S, Josa M, Freixa X, Azqueta M, Mestres C, Mulet J. Mitral insufficiency with a double-orifice mitral valve in an adult patient. J Thorac Cardiovasc Surg. 2007. 134:250–251.