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
نویسندگان
چکیده
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 This is being superseded by valve-sparing aortic root replacement to avoid lifelong anticoagulation.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.
منابع مشابه
AORTIC DISEASE REVIEW SERIES Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms
To cite: Treasure T, Takkenberg JJM, Pepper J. Heart 2014;100:1571– 1576. ABSTRACT Elective root replacement in Marfan syndrome has improved life expectancy in affected patients. Three forms of surgery are now available: total root replacement (TRR) with a valved conduit, valve sparing root replacement (VSRR) and personalised external aortic root support (PEARS) with a macroporous mesh sleeve. ...
متن کاملSurgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms
Elective root replacement in Marfan syndrome has improved life expectancy in affected patients. Three forms of surgery are now available: total root replacement (TRR) with a valved conduit, valve sparing root replacement (VSRR) and personalised external aortic root support (PEARS) with a macroporous mesh sleeve. TRR can be performed irrespective of aortic dimensions and a mechanical replacement...
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Background: The usage of pericardium of patient himself, for support of anastomosis lines may carry benefits in patients undergoing aortic valve replacement with pulmonary autograft (Ross). The aim of the present study was to compare the short term results of aortic valve replacement with pulmonary autograft with routine and offered methods. Methods: In a non-randomized clinical trial, 64 pati...
متن کاملPersonalised External Aortic Root Support (PEARS) Compared with Alternatives for People with Life-Threatening Genetically Determined Aneurysms of the Aortic Root
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...
متن کاملEffect of personalized external aortic root support on aortic root motion and distension in Marfan syndrome patients.
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 o...
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