Long-Term Results of Aortic Root Surgery in Marfan Syndrome Patients: A Single-Center Experience

J Heart Valve Dis. 2017 Jul;26(4):397-404.

Abstract

Background and aim of the study: The study aim was to compare long-term results of Marfan syndrome (MFS) patients affected by aortic root disease undergoing aortic root replacement with the Bentall or David operation.

Methods: Since 1994, a total of 59 patients has been followed at the authors' Marfan Center, having undergone either a Bentall operation (Bentall group, n = 30) or a David operation (David group, n = 29).

Results: No operative mortality was recorded. After 20 years (mean follow up 97 ± 82 months; range 1 to 369 months) no prosthesis-related major bleeding or thromboembolic events had been observed; the 20-year survival was 94 ± 6% in the Bentall group, and 100% in the David group (p = 0.32). Freedom from reintervention for aortic valve dysfunction was 100% in the Bentall group, and 75 ± 13% in the David group (p = 0.04). This inter-group difference became relevant after the first eight-year period of follow-up, and was mainly associated with a particular familiar genetic phenotype involving three out of four reoperated patients. Freedom from all-cause death, myocardial infarction, stroke, prosthetic valve-related complications, and reintervention on any aortic segment was 69 ± 12% in the Bentall group, and 67 ± 14% in the David group (p = 0.33).

Conclusions: The Bentall and David operations are both associated with satisfactory long-term results in MFS patients. The low rate of valve prosthesis-related complications suggested that the Bentall operation would continue to be a standard surgical treatment. The reimplantation technique, adopted for less-dilated aortas, provides satisfactory freedom from reoperation. Careful attention should be paid to the reimplantation technique in patients affected by a serious familiar genetic phenotype.

Publication types

  • Observational Study

MeSH terms

  • Adult
  • Aortic Aneurysm / diagnostic imaging
  • Aortic Aneurysm / mortality
  • Aortic Aneurysm / physiopathology
  • Aortic Aneurysm / surgery*
  • Aortic Valve Insufficiency / pathology
  • Aortic Valve Insufficiency / physiopathology
  • Aortic Valve Insufficiency / surgery*
  • Blood Vessel Prosthesis
  • Blood Vessel Prosthesis Implantation* / adverse effects
  • Blood Vessel Prosthesis Implantation* / instrumentation
  • Blood Vessel Prosthesis Implantation* / mortality
  • Dilatation, Pathologic
  • Disease-Free Survival
  • Female
  • Heart Valve Prosthesis
  • Heart Valve Prosthesis Implantation* / adverse effects
  • Heart Valve Prosthesis Implantation* / instrumentation
  • Heart Valve Prosthesis Implantation* / mortality
  • Humans
  • Kaplan-Meier Estimate
  • Male
  • Marfan Syndrome / diagnostic imaging
  • Marfan Syndrome / mortality
  • Marfan Syndrome / physiopathology
  • Marfan Syndrome / surgery*
  • Middle Aged
  • Proportional Hazards Models
  • Prosthesis Failure
  • Replantation
  • Retrospective Studies
  • Risk Factors
  • Rome
  • Time Factors
  • Treatment Outcome
  • Young Adult