title: Effect of personalized external aortic root support on aortic root motion and distension in Marfan syndrome patients.
abstract: OBJECTIVE
  Personalized external aortic root support (PEARS) is a novel surgical approach with the aim of stabilizing the aortic root size and decreasing risk of dissection in Marfan syndrome patients. A bespoke polymer mesh tailored to each patient's individual aorta shape is produced by modeling and then surgically implanted. The aim of this study is to assess the mechanical effects of PEARS on the aortic root systolic downward motion (an important determinant of aortic wall stress), aortic root distension and on the left ventricle (LV).
  
  
  RESULTS
  A cohort of 27 Marfan patients had a prophylactic PEARS surgery between 2004 and 2012 with 24 having preoperative and follow-up cardiovascular magnetic resonance imaging studies. Systolic downward aortic root motion, aortic root distension, LV volumes/mass and mitral annular systolic excursion before the operation and in the latest follow-up were measured randomly and blinded. After a median follow-up of 50.5 (IQR 25.5-72) months following implantation of PEARS, systolic downward motion of aortic root was significantly decreased (12.6±3.6mm pre-operation vs 7.9±2.9mm latest follow-up, p<0.00001). There was a tendency for a decrease in systolic aortic root distension but this was not significant (median 4.5% vs 2%, p=0.35). There was no significant change in LV volumes, ejection fraction, mass and mitral annular systolic excursion in follow-up.
  
  
  CONCLUSIONS
  PEARS surgery decreases systolic downward aortic root motion which is an important determinant of longitudinal aortic wall stress. Aortic wall distension and Windkessel function are not significantly impaired in the follow-up after implantation of the mesh which is also supported by the lack of deterioration of LV volumes or mass.
authors.affiliation: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK.
authors.forename: Cemil
authors.initials: C
authors.lastname: Izgi
authors.affiliation: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK.
authors.forename: Evangelia
authors.initials: E
authors.lastname: Nyktari
authors.affiliation: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK.
authors.forename: Francisco
authors.initials: F
authors.lastname: Alpendurada
authors.affiliation: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK.
authors.forename: Annina Studer
authors.initials: AS
authors.lastname: Bruengger
authors.affiliation: Department of Cardiac Surgery, Royal Brompton Hospital, London, UK; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital, London, UK.
authors.forename: John
authors.initials: J
authors.lastname: Pepper
authors.affiliation: Clinical Operational Research Unit, University College London, London, UK.
authors.forename: Tom
authors.initials: T
authors.lastname: Treasure
authors.affiliation: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital, London, UK; National Heart and Lung Institute, Imperial College London, London, UK. Electronic address: R.Mohiaddin@rbht.nhs.uk.
authors.forename: Raad
authors.initials: R
authors.lastname: Mohiaddin
mesh_terms: D000328:Adult; D000368:Aged; D001013:Aorta, Thoracic / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D017545:Aortic Aneurysm, Thoracic / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D001807:Blood Vessel Prosthesis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D008875:Middle Aged; D011474:Prosthesis Design; D013318:Stroke Volume; D013526:Surgical Mesh; D016896:Treatment Outcome
keywords: Aneurysm; Aorta; Elasticity; Magnetic resonance imaging; Marfan syndrome; Prevention