J Cardiovasc Ultrasound.  2016 Sep;24(3):247-250. 10.4250/jcu.2016.24.3.247.

Severe Aortic Stenosis Associated with Unicommissural Unicuspid Aortic Valve in a Middle Aged Male

• Kwon HJ
• Park JH
• Kim SS
• Sun BJ
• Jin SA
• Kim JH
• Lee JH
• Choi S
• Jeong JO
• Seong IW

• KMID: 2353104
• DOI: http://doi.org/10.4250/jcu.2016.24.3.247

Abstract

Unicuspid aortic valve (UAV) is an extremely rare form of congenital aortic valvular abnormality. Although UAV shows similar clinical characteristics to bicuspid aortic valve, the clinical symptoms develop at earlier age and progress at a faster pace in UAV. In this report, we are presenting a 42-year-old male with severe aortic stenosis associated with unicommissural UAV. The patients underwent a successful Bentall operation.

Keyword

Aortic stenosis; Severe; Unicuspid aortic valve; Aortic aneurysm

MeSH Terms

Adult
Aortic Aneurysm
Aortic Valve Stenosis*
Aortic Valve*
Bicuspid
Humans
Male*
Middle Aged*

Figure

• 

  Fig. 1 Transthoracic echocardiography at the parasternal short axis view shows eccentric slit-like aortic valve opening (arrows) with heavy calcification in the middle portion (arrowheads, A in end-diastole and B in mid-systole).

• 

  Fig. 2 The computerized tomography of the aorta demonstrates thickened unicuspid aortic valve with eccentric slit-like aortic valve opening (arrow, A) and ascending aorta dilatation up to 50 mm (B).

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  Fig. 3 The surgical specimen reveals unicommissural unicuspid aortic valve with heavy calcification (This photography was acquired from the medical record of the Severance Hospital, Yonsei Medical Center, Yonsei University, Seoul, Korea).

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