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Valvular Disease in Marfan Syndrome: Surgical Considerations and Management

  • Ryan P. Plichta
  • Donald D. Glower
  • G. Chad HughesEmail author
Valvular Heart Disease (TL Kiefer, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Valvular Heart Disease

Abstract

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.

Summary

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.

Keywords

Marfan syndrome Valvular disease Surgical management Connective tissue disorder 

Notes

Compliance with Ethical Standards

Conflict of Interest

Ryan P. Plichta, Donald D. Glower, and G. Chad Hughes declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Ryan P. Plichta
    • 1
  • Donald D. Glower
    • 1
  • G. Chad Hughes
    • 1
    Email author
  1. 1.Division of Thoracic and Cardiovascular SurgeryDuke University Medical CenterDurhamUSA

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