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The Marfan aortic root: time to refine surgical guidelines

Nature Reviews Cardiology volume 6, pages 172–175 (2009)Cite this article

Given the prognostic implications, aortic surgery can be the most important event in the life of a patient with Marfan syndrome, which raises questions about when to operate and which procedure to use. Aortic root dilatation begins in infancy, with maximal rates of expansion between 6 and 14 years of age. Afterwards dilatation slows, but increased aortic radius, decreased wall thickness, and elevated wall stiffness cause mural stress to rise. Although β-blockade can slow the rate of aortic expansion,4 for adults, the threshold for surgery occurs when the aortic sinus diameter reaches 5.0 cm. Svensson and Khitin showed that 43% of patients who experienced aortic dissection during surveillance did so before the aortic sinus reached 5.5 cm and 15% experienced dissection before it reached 5.0 cm.5 Furthermore, the aortas of some patients with an adverse family history dissected before they reached 4.5 cm. Accelerated aortic enlargement at any stage (>2.0 cm per year or >5% per year) should precipitate surgery before the 5.0 cm threshold.

Aortic root replacement with a prosthetic valved conduit was first described in 1968 by Bentall and De Bono,6 and soon set the standard for treatment in Marfan syndrome. Technical details are important in this radical procedure—if the reimplanted coronary buttons are too large (>1 cm), even these will become aneurysmal. In the landmark Johns Hopkins series, Gott et al. described 235 patients with Marfan syndrome who had elective surgery over a 24-year period with no in-hospital mortality.7 The percentages of patients free from thromboembolism, prosthetic valve endocarditis, or further aortic surgery at 20 years follow-up were 93%, 90%, and 74%, respectively. The authors also included 36 patients with aortic dissection, 2 of whom died. Actuarial survival for the entire group (n = 271) was 81% at 10 years and 67% at 20 years.

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Figure 1: A pair of siblings with Marfan syndrome who underwent aortic-root surgery.
Figure 2: Valve-conserving aortic-root replacement by the reimplantation method.

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Acknowledgements

Written consent for publication of Figure 1 was obtained from the patients.

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  1. John Radcliffe Hospital, Headley Way, Headington, Oxford OX3 9DU, UK stephen.westaby@orh.nhs.uk

Authors and Affiliations

  1. S Westaby is Professor of Biomedical Science at the University of Wales, Swansea, and a Consultant Cardiac Surgeon at Oxford Radcliffe National Health Service Trust, Oxford, UK.,

    Stephen Westaby

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Westaby, S. The Marfan aortic root: time to refine surgical guidelines. Nat Rev Cardiol 6, 172–175 (2009). https://doi.org/10.1038/ncpcardio1365

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