title: Manufacturing and placing a bespoke support for the Marfan aortic root: description of the method and technical results and status at one year for the first ten patients.
abstract: Fatal aortic dissection commonly occurs in Marfan syndrome. Prevention currently relies on elective replacement of the aortic root. We are evaluating the placement of a manufactured, bespoke external support derived from a computer aided design in a prospective study. In the first ten patients, measurements were made consistently of the ascending aorta at the level of closure of the aortic valve cusps from magnetic resonance imaging (MRI) studies taken preoperatively and at fixed intervals thereafter. Before and after images were presented for measurement amongst duplicate images of 37 unoperated Marfan patients to permit assessment of intra-observer measurement reproducibility. All images were presented in random sequence to a radiologist unaware of the research question. The largest difference between the preoperative measurement and that made at least one year after surgery was determined. All patients had surgery as planned without complications and were alive at one year. In eight of the ten patients, the largest observed change was a marked reduction in aortic root diameter. The primary objective of this surgery was achieved in each case, reinforcing the ascending aorta whilst leaving the native aortic valve intact and conserving the blood/endothelium interface.
authors.affiliation: Royal Brompton Hospital, London, UK.
authors.forename: John
authors.initials: J
authors.lastname: Pepper
authors.forename: Tal
authors.initials: T
authors.lastname: Golesworthy
authors.forename: Martin
authors.initials: M
authors.lastname: Utley
authors.forename: John
authors.initials: J
authors.lastname: Chan
authors.forename: Skandadas
authors.initials: S
authors.lastname: Ganeshalingam
authors.forename: Michael
authors.initials: M
authors.lastname: Lamperth
authors.forename: Raad
authors.initials: R
authors.lastname: Mohiaddin
authors.forename: Tom
authors.initials: T
authors.lastname: Treasure
mesh_terms: D000293:Adolescent; D000328:Adult; D001014:Aortic Aneurysm / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D001807:Blood Vessel Prosthesis; D019917:Blood Vessel Prosthesis Implantation / Q000009:adverse effects / Q000295:instrumentation*; D017076:Computer-Aided Design; D005260:Female; D006801:Humans; D018810:Magnetic Resonance Angiography; D008297:Male; D008382:Marfan Syndrome / Q000150:complications / Q000473:pathology / Q000601:surgery*; D008875:Middle Aged; D015588:Observer Variation; D011237:Predictive Value of Tests; D011446:Prospective Studies; D011474:Prosthesis Design; D015203:Reproducibility of Results; D013526:Surgical Mesh; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult