Abstract
Aneurysms of the thoracic aorta often have a strong genetic link in their aetiology. The commonest example of this is the Marfan Syndrome. Fatal aortic dissection occurs at a young age in this disease. Prevention relies on elective replacement of the aortic root. The placement of an external root support, tailored to the anatomy of the individual patient has been proposed as a feasible alternative. We have offered this procedure to patients with Marfan syndrome whose aortic root diameter lies between 40 and 55 mm and without aortic regurgitation. By computer-aided design, a model of the individual patient’s aorta was created from cardiac magnetic resonance images and a bespoke external aortic support was manufactured. Comparative measurements were made of the ascending aorta at the level of closure of the aortic valve leaflets from magnetic resonance imaging studies.
Thirty three patients have been operated upon from May 2004 to July 2012, 23 men and 10 women, median age 33 years. All patients are alive and well at the time of last follow-up. Preoperative aortic root diameters were 40–50 mm. All postoperative images were satisfactory with an overall reduction in aortic root dimensions. The operation took half the time of other aortic root surgery, cardiopulmonary bypass was used only in the first patient for 20 min, myocardial ischaemia was not required in any patient, and no post-operative anticoagulation is mandated.
The primary objective of the surgery was achieved, reinforcing the ascending aorta while leaving the native aortic valve intact and conserving the blood/endothelium interface.
On Behalf Of The Exostent Group Of Surgeons: Mario Petrou, Oxford University Hospitals Nhs Trust, Oxford, United Kingdom; Filip Riga, Department Of Cardiac Surgery, University Of Leuven, Leuven, Belgium; Ulrich Rosendhal, Royal Brompton Hospital, London, United Kingdom; And Tom Treasure, CORU, UCL, London, United Kingdom
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Johansson G, Markstrom U, Swedenborg J. Ruptured thoracic aortic aneurysm: a study of incidence and mortality rates. J Vasc Surg. 1995;21:985–8.
Olsson C, Thelin S, Stahle E, Ekbom A, Granath F. Thoracic aortic aneurysm and dissection: increasing prevalence and improved outcomes reported in a nationwide population-based study of more than 14,000 cases from 1987 to 2002. Circulation. 2006;114:2611–8.
Michel J-B, Touat Z, Houard X, Borges LF, Fontaine V, Jondeau G. Chapter 8. New concepts in human abdominal aortic aneurysm pathophysiology: comparison with ascending aortic and other locations. In: Sakalihasan N, Kuivaniemi H, Michel J-B, editors. Aortic aneurysms: new insights into an old problem. Liege: Les Editions de l’Universitie de Liege; 2008. ISBN 9–782874–560613.
Schlatmann TJ, Becker AE. Histologic changes in the normal ageing aorta: implications for dissecting aortic aneurysms. Am J Cardiol. 1977;39:13–20.
Virmani R, Avolio AP, Mergner WJ, Robinowitz M, Herderick EE, Cornhill JF, Guo SY, Liu TH, Ou DY, O’Rourke M. Effect of ageing on aortic morphology in populations with high and low prevalence of hypertension and atherosclerosis. Comparison between occidental and Chinese communities. Am J Pathol. 1991;139:1119–29.
Mizuguchi T, Collod-Beroud G, Akiyama T, Abifadel M, Harada N, Ihara M, Kinoshita A, Yoshiwa K, Junien C, Kajii T, Jondeau G, Ohta T, Kishino T, Furukawa Y, Nakamura Y, Niikawa N, Boileau C, Matsumoto N. Heterozygous TGFBR2 mutations in Marfan syndrome. Nat Genet. 2004;36:855–60.
Loeys BL, Schwarze U, Holm T, Callewaert BL, Thomas GH, Pannu H, De Backer JF, Oswald GL, Symoens S, Manouvrier S, Roberts AF, Faravelli F, Greco MA, Pyeritz RE, Milewicz DM, Coucke PJ, Cameron DE, Braverman AC, Byers PH, De Paepe AM, Dietz HC. Aneurysm syndromes caused by mutations in the TGF-beta receptor. N Engl J Med. 2006;355:788–98.
Malfait F, Symoens S, De Backer J, Hermanns-Le T, Sakalihasan N, Lapiere CM, Coucke P, De Paepe A. Three arginine to cysteine substitutions in the pro-alpha (l)-collagen chain cause Ehlers-Danlos syndrome with a propensity for arterial rupture in early childhood. Hum Mutat. 2007;28:387–95.
Coucke PJ, Willaert A, Wessels MW, Callewaert B, Zoppi N, De Backer J, Fox JE, Mancini GMS, Kambouris M, Gardella R, Facchetti F, Willems PJ, Forsyth R, Dietz HC, Barlati S, Colombi M, Loeys B, De Paepe A. Mutations in the facilitative glucose transporter GLUT 10 alter angiogenesis and cause arterial tortuosity syndrome. Nat Genet. 2006;38:452–7.
Robiscek F, Thubrikar MJ, Cook JW, Fowler B. The congenitally bicuspid aortic valve: how does it function? Why does it fail? Ann Thorac Surg. 2004;77:177–85.
Morgan-Hughes GJ, Roobottom CA, Owens PE, Marshall AJ. Dilatation of the aorta in pure, severe, bicuspid aortic valve stenosis. Am Heart J. 2004;147:736–40.
Braverman AC, Guven H, Beardslee MA. The bicuspid aortic valve. Curr Probl Cardiol. 2005;30:470–522.
Murdoch JL, Walker BA, Halpern BL, Kuzma JW, McKusick VA. Life expectancy and causes of death in the Marfan syndrome. N Engl J Med. 1972;286:804–8.
Bentall H, DeBono A. A technique for complete replacement of the ascending aorta. Thorax. 1968;23:338–9.
Gott VL, Cameron DE, Alejo DE, Greene PS, Shake JG, Caparelli DJ. Aortic root replacement in 271 Marfan patients: a 24-year experience. Ann Thorac Surg. 2002;73:438–43.
Murgatroyd F, Child A, Poloniecki J, Treasure T, Pumphrey C. Does routine echocardiographic measurement of the aortic root diameter predict the risk of aortic dissection in the Marfan syndrome? Eur Heart J. 1991;12:410.
Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE, Eagle KA, Hermann LK, Isselbacher EM, Kazerooni EA, Kouchoukos NT, Lytle BW, Milewicz DM, Reich DL, Sen S, Shinn JA, Svensson LG, Williams DM. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM. Guidelines for the diagnosis and management of patients with thoracic aortic disease: executive summary. Circulation. 2010;121:1544–79.
Benedetto U, Melina G, Takkenberg JJ, Roscitano A, Angeloni E, Sinatra R. Surgical management of aortic root disease in Marfan syndrome: a systematic review and meta-analysis. Heart. 2011;97:955–8.
Gott VL, Greene PS, Alejo DE, Cameron DE, Naftel DC, Miller DC. Replacement of the aortic root in Marfan’s syndrome. N Engl J Med. 1999;340:1307–13.
Fagan A, Pillai R, Radley-Smith R, Yacoub M. Results of new valve conserving operation for treatment of aneurysms or acute dissection of aortic root. Br Heart J. 1983;49:302.
David TE, Feindel CM. An aortic valve-sparing operation for patients with aortic incompetence and aneurysm of the ascending aorta. J Thorac Cardiovasc Surg. 1992;103:617–21.
David TE, Fiendel CM, Webb G, Colman JM, Armstrong S, Maganti M. Long-term results of aortic valve-sparing operations for aortic root aneurysm. J Thorac Cardiovasc Surg. 2006;132:347–54.
Golesworthy T, Lamperth M, Mohiaddin R, Pepper J, Thornton W, Treasure T. The Tailor of Gloucester: a jacket for the Marfan’s aorta. Lancet. 2004;364:1582.
Treasure T. Elective replacement of the aortic root in Marfan’s syndrome. Br Heart J. 1993;69:101–3.
Treasure T, Pepper JR. Aortic root surgery in Marfan syndrome. Heart. 2011;97:951–2.
Pepper J, Golesworthy T, Utley M, Chan J, Ganeshalingam S, Lamperth M, Mohiaddin R, Treasure T. Manufacturing and placing a bespoke support for the Marfan aortic root: description of the method and technical results and status at 1 year for the first ten patients. Interact Cardiovasc Thorac Surg. 2010;10:360–5.
Robicsek F, Thubrikar MJ. Conservative operation in the management of annular dilatation and ascending aortic aneurysm. Ann Thorac Surg. 2004;57:1672–4.
Pepper J, John CK, Gavino J, Golesworthy T, Mohiaddin R, Treasure T. External aortic root support for Marfan syndrome: early clinical results in the first 20 recipients with a bespoke implant. J R Soc Med. 2010;103:370–5.
Allen C, Pepper J. External aortic support for people with Marfan’s syndrome. BMJ. 2010;340:1692.
Min SK, Kenagy RD, Jeanette JP, Clowes AW. Effects of external wrapping and increased blood flow on atrophy of the baboon iliac artery. J Vasc Surg. 2008;47:1039–47.
Kim SY, Martin N, Hsia EC, Pyeritz RE, Albert DA. Management of aortic disease in Marfan syndrome: a decision analysis. Arch Intern Med. 2005;165:749–55.
Niwa K, Siu SC, Webb GD. Progressive aortic root dilatation in adults late after repair of tetralogy of Fallot. Circulation. 2002;106:1374–8.
Tan JL, Gatzoulis MA, Ho SY. Aortic root disease in Tetralogy of Fallot. Curr Opin Cardiol. 2006;21:569–72.
David TE, Omran A, Ivanov J, Armstrong S, de Sa MP, Sonnenberg B, Webb G. Dilation of the pulmonary autograft after the Ross procedure. J Thorac Cardiovasc Surg. 2000;119:210–20.
Brown JW, Ruzmetov M, Shahriari AP, Rodefield MD, Mahomed Y, Turrentine MW. Modification of the Ross aortic valve replacement to prevent late autograft dilatation. Eur J Cardiothorac Surg. 2010;37:1002–7.
Habashi JP, Judge DP, Holm TM, Cohn RD, Loeys BL, Cooper TK. Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome. Science. 2006;312:117–21.
Keane MG, Pyeritz RE. Medical management of Marfan Syndrome. Circulation. 2008;117:2802–13.
Forteza A, Evangelista A, Sanchez V, Teixido G, Garcia D, Sanz P, Gutierrez L, Centeno J, Rodriguez-Palomares J, Cortina J, Garcia-Dorado D. Study of the efficacy and safety of losartan versus atenolol for aortic dilation in patients with Marfan syndrome. Rev Esp Cardiol. 2011;64:492–8.
Acknowledgement
This work was supported by the NIHR Cardiovascular Biomedical research unit at the Royal Brompton Hospital.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2014 Springer-Verlag London
About this chapter
Cite this chapter
Pepper, J.R. (2014). The Exostent Versus Alternative Strategies: The Role of an External Support Sleeve in Aortic Prophylaxis. In: Bonser, R., Pagano, D., Haverich, A., Mascaro, J. (eds) Controversies in Aortic Dissection and Aneurysmal Disease. Springer, London. https://doi.org/10.1007/978-1-4471-5622-2_5
Download citation
DOI: https://doi.org/10.1007/978-1-4471-5622-2_5
Published:
Publisher Name: Springer, London
Print ISBN: 978-1-4471-5621-5
Online ISBN: 978-1-4471-5622-2
eBook Packages: MedicineMedicine (R0)