Frassani R, Gelsomino S, Puricelli C
U.O. Cardiotoracica, Azienda Ospedaliera S. Maria della Misericordia, Udine.
G Ital Cardiol. 1998 Sep;28(9):1017-20.
We report a case of a young woman affected with Marfan's syndrome, with an ascending aortic aneurysm and mild aortic insufficiency, who underwent a valve-sparing operation as described by Tirone David. In many patients with Marfan's syndrome, aortic valve dysfunction is caused by dilatation of the sinotubular junction with distortion of the sinuses of Valsalva, and often with intact aortic valve leaflets. In these patients, if the aortic valve leaflets are normal at echocardiography, a more aggressive approach may be advisable in order to prevent irreversible damage to the aortic valve leaflets. We believe that surgery is indicated when the aortic root increases rapidly, even if it does not reach a diameter of 50 mm, which represents the current indication for asymptomatic patients with ascending aortic aneurysm. This makes it possible to perform a valve-sparing operation instead of the composite replacement of the aortic valve and ascending aorta, which is still considered the standard treatment for these patients. The procedure described here represents an attractive choice for managing aortic root dilatation and annuloaortic ectasia in Marfan patients, with the benefit of avoiding all the well-known complications involved in prosthetic aortic valves.
我们报告一例患有马凡综合征的年轻女性病例,该患者升主动脉瘤合并轻度主动脉瓣关闭不全,接受了蒂罗内·戴维所描述的保留瓣膜手术。在许多马凡综合征患者中,主动脉瓣功能障碍是由窦管交界扩张伴瓦尔萨尔瓦窦变形引起的,且主动脉瓣叶通常完好无损。在这些患者中,如果超声心动图显示主动脉瓣叶正常,为防止主动脉瓣叶发生不可逆损伤,或许可取更积极的治疗方法。我们认为,即使主动脉根部未达到50毫米的直径(这是目前无症状升主动脉瘤患者的手术指征),但当主动脉根部迅速增大时,手术仍是必要的。这样就有可能进行保留瓣膜手术,而非主动脉瓣和升主动脉的复合置换术,后者仍被视为这些患者的标准治疗方法。本文所述手术方法是治疗马凡综合征患者主动脉根部扩张和主动脉瓣环扩张的一个有吸引力的选择,其好处是避免了人工主动脉瓣所涉及的所有已知并发症。