This Article Figures Only Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Yetman, A. T. Articles by McCrindle, B. W. Search for Related Content PubMed PubMed Citation Articles by Yetman, A. T. Articles by McCrindle, B. W.

CLINICAL STUDY: MARFAN SYNDROME

Long-term outcome in patients with Marfan syndrome: is aortic dissection the only cause of sudden death?

Anji T. Yetman, MD^*,*, Renee A. Bornemeier, MD, FACC^* and Brian W. McCrindle, MD, MPH, FACC

^* University of Arkansas for Medical Sciences, Arkansas Children’s Hospital, Little Rock, Arkansas, USA
The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada

Manuscript received April 29, 2002; revised manuscript received September 11, 2002, accepted October 10, 2002.

^* Reprint requests and correspondence: Dr. Anji Yetman, Department of Cardiology, The Children’s Hospital, 1056 East 19th Avenue, B-100, Denver, Colorado 80218, USA.
Yetman.Angela{at}tchden.org

OBJECTIVES: We sought to assess outcomes in a series of young patients with Marfan syndrome and to define the prevalence of ventricular arrhythmias in this patient population.

BACKGROUND: While sudden death is a well-recognized outcome in Marfan syndrome, ventricular arrhythmias are not well described.

METHODS: Patients were followed with echocardiography, electrocardiography, and ambulatory electrocardiography. The prevalence and associated factors for ventricular dysrhythmias were defined.

RESULTS: Seventy patients with Marfan syndrome diagnosed at birth to 52 years were followed for a period of up to 24 years. All patients had cardiovascular involvement and were started on medical therapy. No patient died from aortic dissection, while 4% died from arrhythmias. Ventricular arrhythmias were present in 21% and were associated with increased left ventricular size, mitral valve prolapse, and abnormalities of repolarization.

CONCLUSIONS: Cardiac complications are rare in young patients with Marfan syndrome receiving medical therapy and close clinical follow-up. Sudden death still occurs, and appears more common in patients with a dilated left ventricle. Left ventricular dilation may predispose to alterations of repolarization and fatal ventricular arrhythmias.

Abbreviations and Acronyms   ECG   electrocardiogram   LV   left ventricle   LVED   left ventricular end-diastolic dimension   MR   mitral regurgitation   MVP   mitral valve prolapse

This article has been cited by other articles:

				A Kiotsekoglou, G R Sutherland, J C Moggridge, D K Nassiri, A J Camm, and A H Child The unravelling of primary myocardial impairment in Marfan syndrome by modern echocardiography Heart, October 1, 2009; 95(19): 1561 - 1566. [Abstract] [Full Text] [PDF]

				R. Formigari, G. Michielon, M. C. Digilio, G. Piacentini, A. Carotti, A. Giardini, R. M. Di Donato, and B. Marino Genetic syndromes and congenital heart defects: how is surgical management affected? Eur. J. Cardiothorac. Surg., April 1, 2009; 35(4): 606 - 614. [Abstract] [Full Text] [PDF]

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

				R. E Pyeritz Marfan syndrome: 30 years of research equals 30 years of additional life expectancy Heart, February 1, 2009; 95(3): 173 - 175. [Full Text] [PDF]

				M. G. Keane and R. E. Pyeritz Medical Management of Marfan Syndrome Circulation, May 27, 2008; 117(21): 2802 - 2813. [Full Text] [PDF]

				Y. von Kodolitsch and P. N Robinson Marfan syndrome: an update of genetics, medical and surgical management Heart, June 1, 2007; 93(6): 755 - 760. [Full Text] [PDF]

				B. J. Maron, M. J. Ackerman, R. A. Nishimura, R. E. Pyeritz, J. A. Towbin, and J. E. Udelson Task Force 4: HCM and other cardiomyopathies, mitral valve prolapse, myocarditis, and Marfan syndrome J. Am. Coll. Cardiol., April 19, 2005; 45(8): 1340 - 1345. [Full Text] [PDF]

				B. J. Maron, B. R. Chaitman, M. J. Ackerman, A. Bayes de Luna, D. Corrado, J. E. Crosson, B. J. Deal, D. J. Driscoll, N.A. M. Estes III, C. G. S. Araujo, et al. Recommendations for Physical Activity and Recreational Sports Participation for Young Patients With Genetic Cardiovascular Diseases Circulation, June 8, 2004; 109(22): 2807 - 2816. [Abstract] [Full Text] [PDF]

				B. J. Maron Sudden Death in Young Athletes N. Engl. J. Med., September 11, 2003; 349(11): 1064 - 1075. [Full Text] [PDF]

				D. A. Danford Marfan Syndrome and Sudden Death AAP Grand Rounds, June 1, 2003; 9(6): 66 - 67. [Full Text] [PDF]