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Abstract

A 16-year-old boy with Marfan syndrome was scheduled for open-heart surgery for dilated aortic sinuses of Valsalva. Preoperative cardiac catheterization with left ventricular angiography demonstrated bulging at the junction of the mitral annulus with an appearance like ‘a swim ring’. Transthoracic echocardiography showed severe mitral prolapse with the mitral valve leaflets rising toward the left atrium and stretching of the chordae tendineae. ECG-gated contrast CT revealed that this bulging was not the left ventricle itself but the interspace between the base of the left ventricle and mitral valve leaflets. This bulging disappeared after mitral valve plasty with an annuloplasty ring. This unusual finding might herald chordae tendinae rupture in Marfan syndrome.

Keywords: Marfan syndrome, mitral valve prolapse, left ventriculography, chordae tendinae rupture