Valvular Disease in Marfan Syndrome: Surgical Considerations and Management.
PURPOSE OF REVIEW: 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. RECENT FINDINGS: 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.
Plichta, RP; Glower, DD; Hughes, GC
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