Audenaert Tjorven, De Pauw Michel, François Katrien, De Backer Julie
Department of Cardiology, Ghent University Hospital, Ghent, Belgium.
Department of Cardiac Surgery, Ghent University Hospital, Ghent, Belgium.
BMJ Case Rep. 2015 Oct 16;2015:bcr2015211138. doi: 10.1136/bcr-2015-211138.
Heart transplantation in patients with Marfan syndrome is challenging and raises concerns with regards to the haemodynamic and immunosuppressive-induced effects on the inherently fragile aorta. Most aortic events following transplantation reported so far in the literature occurred in patients with pre-existent distal aortic dissection. We report a case of successful orthotopic heart transplantation in a patient with Marfan syndrome that was complicated by late-onset type B dissection in pre-existing mild and stable distal aortic dilation. Serial aortic imaging revealed progressive growth at the level of the descending thoracic aorta. An open thoracoabdominal aortic repair procedure was successfully performed 6 months after the transplantation.
马凡综合征患者的心脏移植具有挑战性，且会引发关于血流动力学以及免疫抑制对本就脆弱的主动脉所产生影响的担忧。迄今为止，文献中报道的大多数移植后主动脉事件都发生在已有远端主动脉夹层的患者身上。我们报告了一例马凡综合征患者成功进行原位心脏移植的病例，该患者在原有轻度且稳定的远端主动脉扩张基础上并发了迟发性B型夹层。系列主动脉成像显示胸降主动脉水平有进行性增大。移植后6个月成功进行了开放性胸腹主动脉修复手术。
