Miyagi K, Koja K, Kuniyoshi Y, Iha K, Akasaki M, Kusaba A
Second Department of Surgery, Faculty of Medicine, University of the Ryukyus, Okinawa, Japan.
Nihon Kyobu Geka Gakkai Zasshi. 1994 Mar;42(3):423-6.
A 34-year-old woman with Marfan's syndrome had severe heart failure due to annulo-aortic ectasia and aortic insufficiency, which was accompanied also by a dissecting aneurysm (DeBakey type IIIb) that was demonstrated by aortography. 4 days before the operation, sudden progression of the aneurysm to a DeBakey type II, and finally DeBakey type I dissecting aneurysm was seen. The first operation was an extended aortic resection with replacement from the aortic valve to the descending thoracic aorta (level of the 7th thoracic vertebra) using selective cerebral perfusion. The second operation was a replacement of the residual dissecting aorta from the level of the 8th thoracic vertebra to the celiac artery with partial extracorporeal circulation. The postoperative course of the patient was uneventful. Dissecting aneurysm with Marfan's syndrome should be operated as extensively as possible if necessary.
一名34岁的马凡氏综合征女性患者因主动脉瓣环扩张和主动脉瓣关闭不全而出现严重心力衰竭,同时伴有主动脉造影显示的夹层动脉瘤(DeBakey IIIb型)。手术前4天,动脉瘤突然进展为DeBakey II型,最终发展为DeBakey I型夹层动脉瘤。第一次手术是采用选择性脑灌注进行从主动脉瓣到胸降主动脉(第7胸椎水平)的扩大主动脉切除术并置换。第二次手术是在部分体外循环下,从第8胸椎水平到腹腔动脉置换残留的夹层主动脉。患者术后恢复顺利。如有必要,马凡氏综合征合并的夹层动脉瘤应尽可能进行广泛手术。