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J Echocardiogr Vol.6, No.1, 2008 21 A 26-year-old woman was referred to our hospital for further evaluation of a grade three systolic murmur at the apex revealed during routine examination. She was free of symptoms on arrival. Findings at physical examination were as follows: joint laxity, positive wrist and thumb sign, and facial appearance for Marfan syndrome (MFS). However, she did not have striae atrophicae or recurrent or incisional hernias. Transthoracic two-dimensional echocardiogram revealed mitral leaflet thickening and a prolapse of the anterior mitral leaflet (A3 segment) (Figure 1). The ejection fraction was 68.6 % with a left ventricular diameter of 64 mm in diastole and 36 mm in systole. The color Doppler image displayed moderate mitral regurgitation directed toward the posterior portion of the left atrium (Figure 1). However, of the major diagnostic criteria of MFS, we did not observe dilatation of ascending aorta, enlargement of aortic root, or aortic regurgitation [1]. The clinical diagnosis of MFS depends on a combination of major and minor signs defined in the revised 1996 Ghent Nosology [1]. We proceeded to check other major signs. Ophthalmologic evaluation revealed that she did not have any abnormal findings, such as ectopia lentis. No abnormality was observed on chest x-ray films. Furthermore, none of the patient’s first-degree family members has Marfan syndrome. We could find only one major sign (skeletal features) and one minor sign (mitral regurgitation). Finally, we performed magnetic resonance (MR) imaging. T2-weight spin-echo MR images (Figure 2) revealed extensive dural ectasia with significant expansion of the dura which extended into the soft tissues of the lower back and into the presacral space. Taken together, we diagnosed her as having MFS. Dural ectasia is a sensitive and specific marker that can assist in confirmation of a diagnosis of MFS in young patients [2]. On follow-up, our patient was noted to be without complications.

Mitral Valve Prolapse and Dural Ectasia in a Patient With Marfan Syndrome