Division of Thoracic and Cardiovascular Surgery, Duke University Medical Center, DUMC BOX 3051, Durham, NC, 27710, USA.
Curr Cardiol Rep. 2019 Mar 4;21(4):23. doi: 10.1007/s11886-019-1110-3.
Detail the current strategies for the management of valve dysfunction in Marfan syndrome (MFS), understand the limitations of surgical interventions, and delineate the likely direction of future innovations.
Significant advances in both medical and surgical management of MFS have been made over the last 50 years. This has resulted in improved overall outcomes. As MFS patients age, new clinical challenges that were once rare have emerged and can require complex care strategies. Medical management has seen advances and focuses on anti-impulse and molecular-based pharmacotherapy, along with close monitoring with serial imaging to minimize acute aortic dissection risk by selecting appropriate timing of prophylactic surgical intervention with increasing aortic dimensions. Ongoing trials are evaluating other potential drug therapies with the ultimate goal of targeted treatment. Over the last 50 years, significant advances have been made in the understanding and management of MFS. A move to prophylactic surgery for aortopathy and valve disease has progressed from a valve replacement to a valve-sparing strategy in many cases. However, the durability of these repairs is variable and the possibility of reintervention looms.
详细介绍马凡综合征（MFS）中瓣膜功能障碍的当前管理策略，了解手术干预的局限性，并描绘未来创新的可能方向。
在过去的 50 年中，MFS 的医学和手术管理都取得了重大进展。这导致了整体预后的改善。随着 MFS 患者年龄的增长，曾经罕见的新临床挑战已经出现，可能需要复杂的护理策略。医学管理取得了进展，重点是抗冲动和基于分子的药物治疗，以及密切监测，通过对主动脉尺寸进行连续成像，选择适当的预防性手术干预时机，以最大限度地降低急性主动脉夹层风险。正在进行的试验正在评估其他潜在的药物治疗方法，最终目标是进行靶向治疗。在过去的 50 年中，MFS 的理解和管理取得了重大进展。主动脉病变和瓣膜病的预防性手术已从瓣膜置换术发展到许多情况下的瓣膜保留策略。然而，这些修复的耐久性是可变的，再次干预的可能性仍然存在。
