A Case of Aortitis Syndrome Complicated with Incomplete Marfan's Syndrome Operated by the Cabrol Method / 日本心臓血管外科学会雑誌

ABSTRACT

We report a very rare case of annuloaortic ectasia with an etiology of both aortitis syndrome and Marfan's syndrome. A 25-year-old woman showed AAE and AR. Her mother had died of SLE, but there was no Marfan's syndrome in her family. Her eyes were normal but her finger was long enough to show wrist sign and thumb sign. Urgent operation was performed because of her progressive heart failure. The ascending aorta was enlarged and Valsalva sinuses showed asymmetrical dilatation. The Cabrol operation was done with a composite graft of 23mm Medtronic Hall valve and 26mm Gelseal graft. The valve was sutured to the graft for 5mm from the end of graft to minimize the tension for annulus because of the high invidence of valve detachment and leakage in aortitis syndrome. Pathological study showed findings of both aortitis syndrome and Marfan's syndrome. Postoperative aortography showed good valvular function, and the patient is doing well now at 6 months after operation.