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Ann Thorac Surg 2007;83:1067-1074
© 2007 The Society of Thoracic Surgeons

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Original Articles: Cardiovascular

Are Marfan Syndrome and Marfanoid Patients Distinguishable on Long-Term Follow-Up?

^a Center for Aortic Surgery, Marfan Syndrome, and Connective Tissue Disorders Clinic, Cleveland, Ohio
^b Departments of Thoracic and Cardiovascular Surgery, Quantitative Health Sciences, The Cleveland Clinic, Cleveland, Ohio
^c Department of Cardiovascular Medicine, The Cleveland Clinic, Cleveland, Ohio

Accepted for publication October 23, 2006.

^_* Address correspondence to Dr Svensson, Center for Aortic Surgery, Marfan Syndrome, and Connective Tissue Disorders Clinic, The Cleveland Clinic, 9500 Euclid Ave, Desk F24, Cleveland, OH 44195 (Email: svenssl{at}ccf.org).

Background: It is unclear whether late outcome differs for Marfan syndrome and marfanoid patients. Thus, we compared characteristics of Marfan versus marfanoid patients and their survival and requirement for reoperation.

Methods: From 1978 to October 2003, 162 patients with a presumptive diagnosis of Marfan syndrome underwent operation. We recategorized them as confirmed Marfan (n = 122), marfanoid (n = 23), Ehlers-Danlos syndrome (n = 5), or other (n = 12). Patients categorized as marfanoid failed to meet the major criteria of Marfan syndrome. We compared characteristics of Marfan and marfanoid groups and assessed long-term survival and need for reoperation.

Results: Marfan and marfanoid patients had similar demographics (women, 33% versus 39%; age, 39 ± 13 versus 41 ± 12 years; height, 186 ± 12 cm versus 184 ± 9.6 cm), valve pathophysiology (aortic regurgitation, 66% versus 58%; mitral regurgitation, 58% versus 62%), and aortic pathology (dilated, 40% versus 39%; dissected, 17% versus 13%). Overall hospital survival was 99.3% (144/145), and 10-year survival was similar at 82% in the Marfan and 100% in marfanoid groups (p = 0.13). Patients with aortic dissection (p = 0.001) and mitral valve replacement (p = 0.003) were at higher risk of death. Reoperation was more frequent after separate aortic valve–ascending aorta graft operations (p = 0.04), and among taller patients (p = 0.005). Of 24 Marfan patients with David root reimplantations, none has required reoperation.

Conclusions: Marfan and marfanoid patients have similar physical characteristics and postoperative survival, although reoperation was more frequent in Marfan patients. Surgery before occurrence of aortic dissection or mitral valve repair should reduce the risk of reoperation, but taller patients, irrespective of Marfan or gender, are more likely to require reoperation.

This article has been cited by other articles:

				S. Goland and U. Elkayam Cardiovascular Problems in Pregnant Women With Marfan Syndrome Circulation, February 3, 2009; 119(4): 619 - 623. [Full Text] [PDF]

				G. D. Pearson, R. Devereux, B. Loeys, C. Maslen, D. Milewicz, R. Pyeritz, F. Ramirez, D. Rifkin, L. Sakai, L. Svensson, et al. Report of the National Heart, Lung, and Blood Institute and National Marfan Foundation Working Group on Research in Marfan Syndrome and Related Disorders Circulation, August 12, 2008; 118(7): 785 - 791. [Full Text] [PDF]