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      Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms

      review-article

      1 , 2 , 3 , 4

      Heart

      BMJ Publishing Group

      AORTA, GREAT VESSELS AND TRAUMA, CARDIAC SURGERY

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          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. TRR can be performed irrespective of aortic dimensions and a mechanical replacement valve is a secure and near certain means of correcting aortic valve regurgitation but has thromboembolic and bleeding risks. VSRR offers freedom from anticoagulation and attendant risks of bleeding but reoperation for aortic regurgitation runs at 1.3% per annum. A prospective multi-institutional study has found this to be an underestimate of the true rate of valve-related adverse events. PEARS conserves the aortic root anatomy and optimises the chance of maintaining valve function but average follow-up is under 5 years and so the long-term results are yet to be determined. Patients are on average in their 30s and so the cumulative lifetime need for reoperation, and of any valve-related complications, are consequently substantial. With lowering surgical risk of prophylactic root replacement, the threshold for intervention has reduced progressively over 30 years to 4.5 cm and so an increasing number of patients who are not destined to have a dissection are now having root replacement. In evaluation of these three forms of surgery, the number needed to treat to prevent dissection and the balance of net benefit and harm in future patients must be considered.

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          Most cited references27

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          Guidelines on the management of valvular heart disease (version 2012).

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            A technique for complete replacement of the ascending aorta.

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              Life expectancy and causes of death in the Marfan syndrome.

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                Author and article information

                Journal
                Heart
                Heart
                heartjnl
                heart
                Heart
                BMJ Publishing Group (BMA House, Tavistock Square, London, WC1H 9JR )
                1355-6037
                1468-201X
                15 October 2014
                1 July 2014
                : 100
                : 20
                : 1571-1576
                Affiliations
                [1 ]Clinical Operational Research Unit, University College London , London, UK
                [2 ]Department of Cardio-Thoracic Surgery, Erasmus University Medical Center , Rotterdam, The Netherlands
                [3 ]NIHR Cardiovascular BRU, Royal Brompton Hospital , London, UK
                [4 ]Institute of Cardiovascular Medicine and Science (ICMS) , London, UK
                Author notes
                [Correspondence to ] Professor Tom Treasure, Clinical Operational Research Unit, University College London, 4 Taviton Street, London WC1H 0BT, UK; tom.treasure@ 123456gmail.com
                Article
                heartjnl-2013-305132
                10.1136/heartjnl-2013-305132
                4215278
                24986892
                284b4eeb-a402-4056-8f54-726815d3c1f5
                Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions

                This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 3.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/3.0/

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                Categories
                1506
                Review
                Aortic disease review series
                Custom metadata
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                Cardiovascular Medicine

                aorta, great vessels and trauma, cardiac surgery

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