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

摘要

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 This is being superseded by valve-sparing aortic root replacement to avoid lifelong anticoagulation.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.