Personalised external aortic root support (PEARS) in Marfan syndrome: analysis of 1-9 year outcomes by intention-to-treat in a cohort of the first 30 consecutive patients to receive a novel tissue and valve-conserving procedure, compared with the published results of aortic root replacement

Heart. 2014 Jun;100(12):969-75. doi: 10.1136/heartjnl-2013-304913. Epub 2014 Jan 6.

Abstract

Objective: Among people with Marfan syndrome who have a typical aortic root aneurysm, dissection is a characteristic cause of premature death. To pre-empt Type A dissection, composite root replacement with a mechanical valve became the standard of care in the 1980s and 1990s. This is being superseded by valve-sparing aortic root replacement to avoid lifelong anticoagulation. In 2004, a total root and valve-sparing procedure, personalised external aortic support, was introduced. We report here results among the first 30 recipients.

Methods: From cross-sectional digital images, the patient's own aorta is modelled by computer aided design and a replica is made in thermoplastic by rapid prototyping. On this, a personalised support of a macroporous polymer mesh is manufactured. The mesh is positioned around the aorta, closely applied from the aortoventricular junction to beyond the brachiocephalic artery. The operation is performed with a beating heart and usually without cardiopulmonary bypass.

Results: Between 2004 and 2011, 30 patients, median age 28 years (IQR 20-44) had this operation and have been prospectively followed for 1.4-8.8 years by February 2013. During a total of 133 patient-years there were no deaths or cerebrovascular, aortic or valve-related events. These early outcomes are better than published results for the more radical extirpative root replacement operations.

Conclusions: The aortic valve, the root architecture, and the blood/endothelia interface are conserved. The perioperative burden is less and there has been freedom from aortic and valvular events. A prospective comparative study is planned.

Publication types

  • Comparative Study
  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aortic Aneurysm / diagnosis
  • Aortic Aneurysm / etiology
  • Aortic Aneurysm / surgery*
  • Aortic Valve / surgery*
  • Belgium
  • Blood Vessel Prosthesis Implantation / adverse effects
  • Blood Vessel Prosthesis Implantation / instrumentation*
  • Blood Vessel Prosthesis*
  • Computer-Aided Design
  • Female
  • Follow-Up Studies
  • Heart Valve Prosthesis Implantation
  • Humans
  • Intention to Treat Analysis
  • London
  • Magnetic Resonance Angiography
  • Male
  • Marfan Syndrome / complications*
  • Marfan Syndrome / diagnosis
  • Precision Medicine
  • Prospective Studies
  • Prosthesis Design
  • Surgery, Computer-Assisted / adverse effects
  • Surgery, Computer-Assisted / instrumentation*
  • Surgical Mesh
  • Time Factors
  • Treatment Outcome
  • Young Adult