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Surgical treatment of Marfan syndrome and related disorders is all about dealing with uncertainties
  1. Maarten Groenink1,
  2. David R Koolbergen2,3
  1. 1 Heart Centre, Academic Medical Centre, Amsterdam, The Netherlands
  2. 2 Department of Radiology, Academic Medical Centre, Amsterdam, The Netherlands
  3. 3 Department of Cardiothoracic Surgery, Leiden University Medical Centre, Leiden, The Netherlands
  1. Correspondence to Dr Maarten Groenink, Department of Cardiology, Academic Medical Center, Amsterdam 1105AZ, The Netherlands; m.groenink{at}

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Treatment of Marfan syndrome and the guidelines

It should once again be stated that the only true and undisputed treatment of patients with Marfan syndrome and other forms of familial thoracic aortic aneurysm (currently known as heritable thoracic aortic disease (HTAD)) is surgical.1 2 Although guidelines supply us with the threshold values of aortic root aneurysm size for elective surgical treatment in these conditions, they do not specify which surgical procedure should be performed.3 These surgical procedures have evolved from classical aortic root replacement, including valve replacement with a mechanical or biological valve (Bentall procedure) to valve sparing techniques (David and Yacoub procedures) and even external procedures (personalised external aortic root support (PEARS) procedure) performed off pump, where the aortic root is left in place and reinforced by a sleeve from the outside.4 Moreover, as the newer valve sparing procedures are sometimes considered less invasive or less influential on daily postsurgical life, they may influence timing of elective aortic (root) surgery, relatively independent of the aneurysm size thresholds mentioned in the guidelines. Anticipating the development that transcatheter valve replacement techniques may become available in the future to replace valves that eventually may fail after a valve sparing procedure,5 the discussion about the timing and choice of surgical procedure may become even more complex as well as uncertain. Although aortic root …

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  • MG and DRK contributed equally.

  • Competing interests None declared.

  • Provenance and peer review Commissioned; internally peer reviewed.

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