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Aortic Dissection in Patients with Disorders of Connective Tissue

  • Florian S. SchoenhoffEmail author
  • Thierry P. Carrel
Chapter

Abstract

Over the past decade, the medical community has slowly accepted the idea that patients presenting with aortic aneurysms and dissections are part of a wide spectrum of genetically mediated diseases that present in syndromic as well as non-syndromic forms. Marfan syndrome (MFS) has long been the only seriously considered differential diagnosis in terms of a heritable disorder of connective tissue in patients with aortic aneurysm.

While growing clinical experience in the treatment of patients with connective tissue disorders has improved surgical management and decision-making, the aorta has emerged as a distinct organ with specific susceptibilities and reactions to pathological stimuli. The spectrum of heritable disorders of aortic disease has certainly become broader necessitating a structured approach to this patient population.

Keywords

Marfan Loeys-Dietz Ehlers-Danlos Connective tissue disorder Aortic aneurysm Aortic dissection 

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Copyright information

© Springer-Verlag GmbH Austria, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Cardiovascular SurgeryUniversity Hospital BernBerneSwitzerland

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