title: External aortic root support for Marfan syndrome: early clinical results in the first 20 recipients with a bespoke implant.
abstract: OBJECTIVE
  Fatal aortic dissection occurs at young age in Marfan syndrome. Prevention relies on elective replacement of the aortic root. The placement of an external aortic root support, tailored to the anatomy of the individual patient has been proposed as a feasible alternative. DESIGN, SETTING AND MAIN OUTCOME MEASURES: External aortic root support was offered to patients with Marfan syndrome with aortic root diameter of 40-55 mm and without aortic regurgitation. By computer-aided design, a model of the individual patient's aorta was created from cardiac magnetic resonance images and a bespoke external aortic support was manufactured. Comparative measurements were made of the ascending aorta at the level of closure of the aortic valve cusps from magnetic resonance imaging studies taken preoperatively, at first follow-up, and at most recent follow-up. For patients having aortic root surgery at the same institution, in the same time frame as the first 10 patients, clinical data were retrieved on Marfan and other patients having aortic root replacement to serve as a reference data.
  
  
  RESULTS
  Twenty patients were operated upon from May 2004 to October 2009, 13 men and 7 women, median age 33 years. All 20 patients are alive and well at the time of last follow-up. Preoperative aortic diameters were 40-54 mm. All postoperative images were satisfactory with an overall reduction in aortic root dimensions. The surgery took half the time of other aortic root surgery. Cardiopulmonary bypass was used only in the first patient, myocardial ischemia was not required in any patient, and no postoperative anticoagulation is mandated.
  
  
  CONCLUSIONS
  The primary objective of this surgery was fully achieved in 19 of the 20 patients, reinforcing the ascending aorta while leaving the native aortic valve intact and conserving the blood/endothelium interface.
authors.affiliation: Royal Brompton Hospital London SW3 6NP, UK.
authors.forename: John
authors.initials: J
authors.lastname: Pepper
authors.forename: Km
authors.initials: K
authors.lastname: John Chan
authors.forename: Jemyrr
authors.initials: J
authors.lastname: Gavino
authors.forename: Tal
authors.initials: T
authors.lastname: Golesworthy
authors.forename: Raad
authors.initials: R
authors.lastname: Mohiaddin
authors.forename: Tom
authors.initials: T
authors.lastname: Treasure
mesh_terms: D000293:Adolescent; D000328:Adult; D000784:Aortic Dissection / Q000517:prevention & control*; D001011:Aorta / Q000473:pathology / Q000601:surgery*; D001014:Aortic Aneurysm / Q000517:prevention & control*; D019917:Blood Vessel Prosthesis Implantation / Q000379:methods*; D017076:Computer-Aided Design; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications / Q000473:pathology / Q000601:surgery*; D008875:Middle Aged; D011446:Prospective Studies; D016896:Treatment Outcome; D055815:Young Adult
references.citation: N Engl J Med. 1999 Nov 4;341(19):1473-4
references.pmid: 10577102
references.citation: Am J Cardiol. 2004 Aug 1;94(3):400-2
references.pmid: 15276119
references.citation: Eur J Cardiothorac Surg. 2009 Jan;35(1):77-82
references.pmid: 18952452
references.citation: Br Heart J. 1993 Feb;69(2):101-3
references.pmid: 8435232
references.citation: Interact Cardiovasc Thorac Surg. 2010 Mar;10(3):353-5
references.pmid: 20118123
references.citation: Ann Thorac Surg. 1994 Jun;57(6):1672-4
references.pmid: 8010829
references.citation: J Thorac Cardiovasc Surg. 1995 Feb;109(2):345-51; discussion 351-2
references.pmid: 7853886
references.citation: Lancet. 2004 Oct 30-Nov 5;364(9445):1582
references.pmid: 15519627
references.citation: J Thorac Cardiovasc Surg. 1998 May;115(5):1080-90
references.pmid: 9605078
references.citation: Ann Thorac Surg. 2002 Feb;73(2):438-43
references.pmid: 11845856
references.citation: Interact Cardiovasc Thorac Surg. 2010 Mar;10(3):360-5
references.pmid: 20007995
references.citation: Thorax. 1968 Jul;23(4):338-9
references.pmid: 5664694