{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T04:23:00Z","timestamp":1760242980968,"version":"build-2065373602"},"reference-count":47,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2015,1,15]],"date-time":"2015-01-15T00:00:00Z","timestamp":1421280000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diseases"],"abstract":"<jats:p>Personalised external aortic support was first proposed in 2000 by Tal Golesworthy, an engineer with familial Marfan syndrome and an aortic root aneurysm. After putting together a research and development team, and finding a surgeon to take on the challenge to join him in this innovative approach, he was central to the manufacture of the device, custom made for his own aorta. He was the patient for the \u2018first in man\u2019 operation in 2004. Ten years later he is well and 45 other people have had their own personalised device implanted. In this account, the stepwise record of proof of principle, comparative quantification of the surgical and perioperative requirements, 10 years of results, and development and research plans for the future are presented.<\/jats:p>","DOI":"10.3390\/diseases3010002","type":"journal-article","created":{"date-parts":[[2015,1,15]],"date-time":"2015-01-15T09:54:54Z","timestamp":1421315694000},"page":"2-14","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Personalised External Aortic Root Support (PEARS) Compared with Alternatives for People with Life-Threatening Genetically Determined Aneurysms of the Aortic Root"],"prefix":"10.3390","volume":"3","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9358-7610","authenticated-orcid":false,"given":"Tom","family":"Treasure","sequence":"first","affiliation":[{"name":"Clinical Operational Research Unit, Department of Mathematics, University College London, Gower Street, London WC1E 6BT, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John","family":"Pepper","sequence":"additional","affiliation":[{"name":"National Institute for Health Research (NIHR) Cardiovascular Biomedical Research Unit (BRU), Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2015,1,15]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1136\/heartjnl-2011-301017","article-title":"External aortic root support: NICE guidance","volume":"98","author":"Treasure","year":"2012","journal-title":"Heart"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1582","DOI":"10.1016\/S0140-6736(04)17308-X","article-title":"A jacket for the Marfan\u2019s aorta","volume":"364","author":"Golesworthy","year":"2004","journal-title":"Lancet"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1089","DOI":"10.1016\/S0140-6736(09)61083-7","article-title":"Evaluation and stages of surgical innovations","volume":"374","author":"Barkun","year":"2009","journal-title":"Lancet"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1097","DOI":"10.1016\/S0140-6736(09)61086-2","article-title":"Challenges in evaluating surgical innovation","volume":"374","author":"Ergina","year":"2009","journal-title":"Lancet"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1105","DOI":"10.1016\/S0140-6736(09)61116-8","article-title":"No surgical innovation without evaluation: the IDEAL recommendations","volume":"374","author":"McCulloch","year":"2009","journal-title":"Lancet"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"f2820","DOI":"10.1136\/bmj.f2820","article-title":"IDEAL framework for surgical innovation 3: Randomised controlled trials in the assessment stage and evaluations in the long term study stage","volume":"346","author":"Cook","year":"2013","journal-title":"BMJ"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"f3011","DOI":"10.1136\/bmj.f3011","article-title":"IDEAL framework for surgical innovation 2: observational studies in the exploration and assessment stages","volume":"346","author":"Ergina","year":"2013","journal-title":"BMJ"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"f3012","DOI":"10.1136\/bmj.f3012","article-title":"IDEAL framework for surgical innovation 1: The idea and development stages","volume":"346","author":"McCulloch","year":"2013","journal-title":"BMJ"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1136\/jmg.31.1.51","article-title":"Ascertainment and severity of Marfan syndrome in a Scottish population","volume":"31","author":"Gray","year":"1994","journal-title":"J. Med. Genet."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1016\/j.mayocp.2013.08.022","article-title":"Epidemiological profile of Marfan syndrome in a general population: a national database study","volume":"89","author":"Chiu","year":"2014","journal-title":"Mayo Clin. Proc."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"804","DOI":"10.1056\/NEJM197204132861502","article-title":"Life expectancy and causes of death in the Marfan syndrome","volume":"286","author":"Murdoch","year":"1972","journal-title":"N. Engl. J. Med."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1965","DOI":"10.1016\/S0140-6736(05)67789-6","article-title":"Marfan's syndrome","volume":"366","author":"Judge","year":"2005","journal-title":"Lancet"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1016\/S0002-9149(00)80066-1","article-title":"Life expectancy in the Marfan syndrome","volume":"75","author":"Silverman","year":"1995","journal-title":"Am. J. Cardiol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"2451","DOI":"10.1093\/eurheartj\/ehs109","article-title":"Guidelines on the management of valvular heart disease (version 2012)","volume":"33","author":"Vahanian","year":"2012","journal-title":"Eur. Heart J."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1254","DOI":"10.1097\/JTO.0b013e3181ae26ae","article-title":"The Mesothelioma and Radical surgery randomized controlled trial: the Mars feasibility study","volume":"4","author":"Treasure","year":"2009","journal-title":"J. Thorac. Oncol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1136\/thoraxjnl-2011-200015","article-title":"Pulmonary metastasectomy in colorectal cancer: the PulMiCC trial","volume":"67","author":"Treasure","year":"2012","journal-title":"Thorax"},{"key":"ref_17","doi-asserted-by":"crossref","unstructured":"Treasure, T., Takkenberg, J.J., and Pepper, J. (2014). Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms. Heart.","DOI":"10.1136\/heartjnl-2013-305132"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"338","DOI":"10.1136\/thx.23.4.338","article-title":"A technique for complete replacement of the ascending aorta","volume":"23","author":"Bentall","year":"1968","journal-title":"Thorax"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1510\/icvts.2010.232611","article-title":"The evolution of aortic root surgery for Marfan syndrome","volume":"10","author":"Treasure","year":"2010","journal-title":"Interact. Cardiovasc. Thorac. Surg."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"955","DOI":"10.1136\/hrt.2010.210286","article-title":"Surgical management of aortic root disease in Marfan syndrome: A systematic review and meta-analysis","volume":"97","author":"Benedetto","year":"2011","journal-title":"Heart"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"969","DOI":"10.1136\/heartjnl-2013-304913","article-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","volume":"100","author":"Treasure","year":"2014","journal-title":"Heart"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1016\/S0022-5223(19)37624-X","article-title":"Surgical management of patients with the Marfan syndrome and dilatation of the ascending aorta","volume":"81","author":"McDonald","year":"1981","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1070","DOI":"10.1056\/NEJM198604243141702","article-title":"Surgical treatment of aneurysms of the ascending aorta in the Marfan syndrome. Results of composite-graft repair in 50 patients","volume":"314","author":"Gott","year":"1986","journal-title":"N. Engl. J. Med."},{"key":"ref_24","first-page":"302","article-title":"Results of new valve conserving operation for treatment of aneurysms or acute dissection of aortic root. (Abstract)","volume":"49","author":"Fagan","year":"1983","journal-title":"Br. Heart J."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1080","DOI":"10.1016\/S0022-5223(98)70408-8","article-title":"Late results of a valve-preserving operation in patients with aneurysms of the ascending aorta and root","volume":"115","author":"Yacoub","year":"1998","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"II29","DOI":"10.1161\/circ.100.suppl_2.Ii-29","article-title":"Early and long-term results of a valve-sparing operation for Marfan syndrome","volume":"100","author":"Birks","year":"1999","journal-title":"Circulation"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1067\/mtc.2001.112935","article-title":"Results of aortic valve-sparing operations","volume":"122","author":"David","year":"2001","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"961","DOI":"10.1016\/j.jtcvs.2005.05.031","article-title":"For everything there is a season","volume":"130","author":"David","year":"2005","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"859","DOI":"10.1016\/j.jtcvs.2009.06.014","article-title":"Long-term results of aortic valve-sparing operations in patients with Marfan syndrome","volume":"138","author":"David","year":"2009","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_30","first-page":"56","article-title":"Long-term results after aortic valve-sparing operation (David I)","volume":"41","author":"Shrestha","year":"2012","journal-title":"Eur. J. Cardiothorac. Surg."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1758","DOI":"10.1016\/j.jtcvs.2014.02.021","article-title":"Early and 1-year outcomes of aortic root surgery in patients with Marfan syndrome: A prospective, multicenter, comparative study","volume":"147","author":"Coselli","year":"2014","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_32","unstructured":"Fosbraey, J. The psychosocial impact of personalised external aortic root support surgery in Marfan syndrome. Available from: http:\/\/www.marfanaorticrootsupport.org\/images\/downloads\/The_psychosocial_impact_of_personalised_external_aortic_root_support_surgery_in_Marfan_syndrome.pdf."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1016\/j.jtcvs.2008.09.028","article-title":"Patient outcome after aortic valve replacement with a mechanical or biological prosthesis: Weighing lifetime anticoagulant-related event risk against reoperation risk","volume":"137","author":"Kappetein","year":"2009","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1161\/CIRCULATIONAHA.110.947341","article-title":"Survival comparison of the Ross procedure and mechanical valve replacement with optimal self-management anticoagulation therapy: Propensity-matched cohort study","volume":"123","author":"Mokhles","year":"2011","journal-title":"Circulation"},{"key":"ref_35","doi-asserted-by":"crossref","unstructured":"Pepper, J., Petrou, M., Rega, F., Rosendahl, U., Golesworthy, T., and Treasure, T. (2013). Implantation of an individually computer-designed and manufactured external support for the Marfan aortic root. Multimedia Manual Cardio-Thoracic Surg.","DOI":"10.1093\/mmcts\/mmt004"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1758","DOI":"10.1016\/j.jtcvs.2014.02.021","article-title":"Early and 1-year outcomes of aortic root surgery in patients with Marfan syndrome: A prospective, multicenter, comparative study","volume":"147","author":"Coselli","year":"2014","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"360","DOI":"10.1510\/icvts.2009.220319","article-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","volume":"10","author":"Pepper","year":"2010","journal-title":"Interact. Cardiovasc. Thorac. Surg."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"e000725","DOI":"10.1136\/bmjopen-2011-000725","article-title":"A method for early evaluation of a recently introduced technology by deriving a comparative group from existing clinical data: A case study in external support of the Marfan aortic root","volume":"2","author":"Treasure","year":"2012","journal-title":"BMJ Open"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"c1692","DOI":"10.1136\/bmj.c1692","article-title":"External aortic support for people with Marfan's syndrome","volume":"340","author":"Allen","year":"2010","journal-title":"BMJ"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"S780","DOI":"10.1016\/j.athoracsur.2006.10.086","article-title":"Long-term experience of girdling the ascending aorta with Dacron mesh as definitive treatment for aneurysmal dilation","volume":"83","author":"Cohen","year":"2007","journal-title":"Ann. Thorac. Surg."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"334","DOI":"10.1093\/icvts\/ivt165","article-title":"External aortic root support: A histological and mechanical study in sheep","volume":"17","author":"Verbrugghe","year":"2013","journal-title":"Interact. Cardiovasc. Thorac. Surg."},{"key":"ref_42","doi-asserted-by":"crossref","unstructured":"Pepper, J., Goddard, M., Mohiaddin, R., and Treasure, T. (2014). Histology of a Marfan aorta 4.5 years after personalized external aortic root support. Eur. J. Cardio-Thoracic Surg.","DOI":"10.1093\/ejcts\/ezu415"},{"key":"ref_43","first-page":"215","article-title":"Prophylactic surgery of the aortic root in Marfan Syndrome: Reconsieration of the ddecision making process in the era of customised external aortic root support","volume":"18","author":"Treasure","year":"2011","journal-title":"Ital. J. Vasc. Endovasc. Surg."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1758","DOI":"10.1016\/j.jtcvs.2014.02.021","article-title":"Early and 1-year outcomes of aortic root surgery in patients with Marfan syndrome: A prospective, multicenter, comparative study","volume":"147","author":"Coselli","year":"2014","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1258\/jrsm.2011.110307","article-title":"GRIST: Growing recruitment in interventional and surgical trials","volume":"105","author":"Treasure","year":"2012","journal-title":"J R. Soc. Med."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"748","DOI":"10.1016\/j.jtcvs.2013.06.025","article-title":"Pulmonary metastasectomy for colorectal cancer: Making the case for a randomized controlled trial in the zone of uncertainty","volume":"146","author":"Fiorentino","year":"2013","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1129","DOI":"10.1016\/j.athoracsur.2013.05.070","article-title":"Pulmonary metastasectomy: Are observational studies sufficient evidence for effectiveness?","volume":"96","author":"Fiorentino","year":"2013","journal-title":"Ann. Thorac. Surg."}],"container-title":["Diseases"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2079-9721\/3\/1\/2\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T20:41:41Z","timestamp":1760215301000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2079-9721\/3\/1\/2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,1,15]]},"references-count":47,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2015,3]]}},"alternative-id":["diseases3010002"],"URL":"https:\/\/doi.org\/10.3390\/diseases3010002","relation":{},"ISSN":["2079-9721"],"issn-type":[{"type":"electronic","value":"2079-9721"}],"subject":[],"published":{"date-parts":[[2015,1,15]]}}}