J Cardiovasc Ultrasound.  2016 Jun;24(2):168-169. 10.4250/jcu.2016.24.2.168.

Rare Case of Unileaflet Mitral Valve

• Shah J
• Jain T
• Shah S
• Mawri S
• Ananthasubramaniam K

• KMID: 2308708
• DOI: http://doi.org/10.4250/jcu.2016.24.2.168

Abstract

Unileaflet mitral valve is the rarest of the congenital mitral valve anomalies and is usually life threatening in infancy due to severe mitral regurgitation (MR). In most asymptomatic individuals, it is mostly due to hypoplastic posterior mitral leaflet. We present a 22-year-old male with palpitations, who was found to have an echocardiogram revealing an elongated anterior mitral valve leaflet with severely hypoplastic posterior mitral valve leaflet appearing as a unileaflet mitral valve without MR. Our case is one of the 11 reported cases in the literature so far. We hereby review those cases and conclude that these patients are likely to be at risk of developing worsening MR later in their lives.

Keyword

Congenital mitral valve anomaly; Unileaflet mitral valve; Mitral regurgitation

MeSH Terms

Humans
Male
Mitral Valve Insufficiency
Mitral Valve*
Young Adult

Figure

• 

  Fig. 1 M mode of the mitral valve showing loss of E to F slope and anterior mitral valve hypertrophy with no contribution from the posterior mitral valve.

• 

  Fig. 2 Transthoracic echocardiogram color Doppler of the parasternal long axis: no evidence of mitral regurgitation.

• 

  Fig. 3 Transthoracic echocardiogram parasternal long-axis view: the anterior mitral valve leaflet was markedly elongated, mildly thickened, and occupied the entire closure line of the mitral annulus with no contribution of the hypoplastic posterior leaflet.

Reference

1. Espinola-Zavaleta N, Vargas-Barrón J, Keirns C, Rivera G, Romero-Cárdenas A, Roldán J, Attie F. Three-dimensional echocardiography in congenital malformations of the mitral valve. J Am Soc Echocardiogr. 2002; 15:468–472.

2. Bär H, Siegmund A, Wolf D, Hardt S, Katus HA, Mereles D. Prevalence of asymptomatic mitral valve malformations. Clin Res Cardiol. 2009; 98:305–309.

3. Bezgin T, Elveran A, Karagöz A, Çanga Y, Yılmaz F. Mitral valve with a single leaflet. Turk Kardiyol Dern Ars. 2014; 42:80–82.

4. Caciolli S, Gelsomino S, Fradella G, Bevilacqua S, Favilli S, Gensini GF. Severe hypoplasia of the posterior mitral leaflet. Ann Thorac Surg. 2008; 86:1978–1979.

5. Kanagala P, Baker S, Green L, Houghton AR. Functionally uni-leaflet mitral valves in a family: a case series. Eur J Echocardiogr. 2010; 11:E27.

6. Ozkan H, Tiryakioglu O, Cetinkaya AS, Uyanik EC, Bozat T. Agenesis of the mitral posterior leaflet in elderly. Ann Thorac Surg. 2014; 97:319–321.

7. Candan O, Guler A, Aung SM, Gecmen C, Karabay CY, Yildiz M. Uni-leaflet mitral valve. Eur J Echocardiogr. 2011; 12:640.