Di Eusanio Marco, Berretta Paolo, Folesani Gianluca, Di Bartolomeo Roberto
Dipartimento di Cardiochirurgia, Policlinico S. Orsola-Malpighi, Università degli Studi, Bologna, Italy.
G Ital Cardiol (Rome). 2013 Jul-Aug;14(7-8):538-47. doi: 10.1714/1308.14462.
Aortic disease is the most life-threatening complication of Marfan syndrome. Over the last decades, improved medical management and surgical results of prophylactic aortic interventions on the aortic root have dramatically increased expectancy of life in Marfan syndrome patients. As a result, the number of Marfan syndrome patients requiring secondary interventions on the thoracic or thoraco-abdominal aorta due to development of aortic disease or new type B dissection, has substantially increased. In this setting, open surgical interventions represent the treatment of choice. Nevertheless, the available literature, although restricted to small case series, indicates that endovascular repair is a feasible treatment option leading to satisfactory short-term results and may provide a bridging role to definitive open reconstruction. The aim of this paper was to review surgical and endovascular outcomes of aortic disease in Marfan syndrome.
主动脉疾病是马凡综合征最危及生命的并发症。在过去几十年中,主动脉根部预防性主动脉干预的医疗管理和手术效果得到改善,显著提高了马凡综合征患者的预期寿命。因此,由于主动脉疾病的发展或新型B型夹层而需要对胸主动脉或胸腹主动脉进行二次干预的马凡综合征患者数量大幅增加。在这种情况下,开放手术干预是首选治疗方法。然而,现有文献虽然限于小病例系列,但表明血管内修复是一种可行的治疗选择,可带来令人满意的短期效果,并且可能为确定性开放重建起到桥梁作用。本文的目的是回顾马凡综合征主动脉疾病的手术和血管内治疗结果。