Aortic root replacement in 372 Marfan patients: evolution of operative repair over 30 years

Duke E Cameron1, Diane E Alejo, Nishant D Patel

  • 1Division of Cardiac Surgery, The Johns Hopkins Medical Institutions, Baltimore, Maryland 21287, USA. dcameron@jhmi.edu

Abstract

Insights

Prophylactic aortic root replacement in Marfan syndrome patients is safe and prevents aortic catastrophe. While valve-sparing techniques show promise, the Bentall procedure remains more durable for aortic root replacement.

Area of Science:

  • Cardiovascular Surgery
  • Genetics
  • Thoracic Surgery

Background:

  • Marfan syndrome is a genetic disorder affecting connective tissue, predisposing patients to aortic root dilation and dissection.
  • Aortic root replacement (ARR) is a critical intervention for Marfan syndrome patients to prevent life-threatening aortic events.
  • Evolution of surgical techniques for ARR in Marfan syndrome necessitates review of long-term outcomes.

Purpose of the Study:

  • To review the evolution of practice and late results of aortic root replacement (ARR) in Marfan syndrome patients.
  • To compare the outcomes of different ARR techniques, including Bentall composite graft, valve-sparing ARR, homografts, and xenografts.
  • To assess the safety and efficacy of prophylactic ascending aorta replacement in Marfan syndrome.

Main Methods:

  • Retrospective clinical review of Marfan syndrome patients who underwent aortic root replacement (ARR) at a single institution.
  • Data collection included hospital records and telephone follow-up for long-term outcomes.
  • Surgical procedures analyzed: Bentall composite graft, valve-sparing ARR (David II remodeling, David I reimplantation), homografts, and porcine xenografts.

Main Results:

  • Between 1976 and 2006, 372 Marfan syndrome patients underwent ARR. The Bentall graft was predominant initially, shifting towards valve-sparing procedures later.
  • No operative or hospital mortality occurred in elective repairs. Urgent/emergent repairs had a 4.4% mortality rate.
  • Late deaths (74) were primarily due to aortic dissection/rupture (10) and arrhythmia (9). Valve-sparing procedures showed promise but were less durable than the Bentall graft, with some requiring re-operation for aortic insufficiency.

Conclusions:

  • Prophylactic surgical replacement of the ascending aorta in Marfan syndrome patients offers low operative risk and effectively prevents aortic catastrophe.
  • Valve-sparing procedures, especially reimplantation techniques with Valsalva grafts, are promising but require further evaluation for long-term durability compared to the Bentall procedure.
  • Continued monitoring and refinement of surgical strategies are essential for optimizing outcomes in Marfan syndrome patients undergoing aortic root replacement.

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