title: 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.
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 This is being superseded by valve-sparing aortic root replacement to avoid lifelong anticoagulation.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.
authors.affiliation: Clinical Operational Research Unit, UCL, London, UK.
authors.forename: Tom
authors.initials: T
authors.lastname: Treasure
authors.affiliation: Department of CardioThoracic Surgery, Erasmus University Medical Center, Rotterdam, Netherlands.
authors.forename: Johanna J M
authors.initials: JJ
authors.lastname: Takkenberg
authors.affiliation: Exstent Ltd, Tewkesbury, UK.
authors.forename: Tal
authors.initials: T
authors.lastname: Golesworthy
authors.affiliation: Department of Cardiac Surgery, University Hospitals Leuven, Leuven, Belgium.
authors.forename: Filip
authors.initials: F
authors.lastname: Rega
authors.affiliation: John Radcliffe Hospital, Oxford, UK.
authors.forename: Mario
authors.initials: M
authors.lastname: Petrou
authors.affiliation: Department of Cardiac Surgery, Royal Brompton Hospital, London, UK.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Rosendahl
authors.affiliation: Department of Cardiac MRI, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College London, London, UK.
authors.forename: Raad
authors.initials: R
authors.lastname: Mohiaddin
authors.affiliation: Department of Radiology, Royal Brompton Hospital, London, UK.
authors.forename: Michael
authors.initials: M
authors.lastname: Rubens
authors.affiliation: Exstent Ltd, Tewkesbury, UK.
authors.forename: Warren
authors.initials: W
authors.lastname: Thornton
authors.affiliation: CTEU, Royal Brompton Hospital, London, UK.
authors.forename: Belinda
authors.initials: B
authors.lastname: Lees
authors.affiliation: NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital, London, UK.
authors.forename: John
authors.initials: J
authors.lastname: Pepper
mesh_terms: D000328:Adult; D001014:Aortic Aneurysm / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D001021:Aortic Valve / Q000601:surgery*; D001530:Belgium; D001807:Blood Vessel Prosthesis; D019917:Blood Vessel Prosthesis Implantation / Q000009:adverse effects / Q000295:instrumentation*; D017076:Computer-Aided Design; D005260:Female; D005500:Follow-Up Studies; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D057194:Intention to Treat Analysis; D008131:London; D018810:Magnetic Resonance Angiography; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000175:diagnosis; D057285:Precision Medicine; D011446:Prospective Studies; D011474:Prosthesis Design; D025321:Surgery, Computer-Assisted / Q000009:adverse effects / Q000295:instrumentation*; D013526:Surgical Mesh; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult