HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Baumgartner, D. Articles by Hammerer, I. Search for Related Content PubMed PubMed Citation Articles by Baumgartner, D. Articles by Hammerer, I. Related Collections Cardiac - physiology Congenital - acyanotic Great vessels

J Thorac Cardiovasc Surg 2005;129:730-739
© 2005 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Diagnostic power of aortic elastic properties in young patients with Marfan syndrome

^a Department of Pediatric Cardiology, Innsbruck Medical University, Innsbruck, Austria
^b Research Group for Biomedical Data Mining, Institute for Information Systems, University for Health Sciences, Medical Informatics and Technology, Innsbruck, Austria
^c Division of Metabolism and Molecular Pediatrics, University Children’s Hospital, Zurich, Switzerland
^d Institute of Medical Genetics, University of Zurich, Schwerzenbach, Switzerland
^e Institute of Medical Biology and Human Genetics, Innsbruck Medical University, Innsbruck, Austria
^f Department of Ophthalmology, Innsbruck Medical University, Innsbruck, Austria
^g Department of Orthopedics, Innsbruck Medical University, Innsbruck, Austria
^h Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center, Munich, Germany

Received for publication March 4, 2004; revisions received June 21, 2004; accepted for publication July 8, 2004.

^_* Address for reprints: Daniela Baumgartner, MD, Department of Pediatric Cardiology, Innsbruck Medical University, Anichstr 35, A-6020 Innsbruck, Austria (E-mail: Daniela.Baumgartner{at}aon.at).

BACKGROUND: In patients with Marfan syndrome, progressive aortic dilation implicates a still-unpredictable risk of life-threatening aortic dissection and rupture. We sought to quantify aortic wall dysfunction noninvasively, determine the diagnostic power of various aortic parameters, and establish a diagnostic model for the early detection of aortic abnormalities associated with Marfan syndrome.

METHODS: In 19 patients with Marfan syndrome (age, 17.7 ± 9.5 years) and 19 age- and sex-matched healthy control subjects, computerized ascending and abdominal aortic wall contour analysis with continuous determination of aortic diameters was performed out of transthoracic M-mode echocardiographic tracings. After simultaneous oscillometric blood pressure measurement, aortic elastic properties were determined automatically.

RESULTS: The following ascending aortic elastic parameters showed statistically significant differences between the Marfan group and the control group: (1) decreased aortic distensibility (P < .001), (2) increased wall stiffness index (P < .01), (3) decreased systolic diameter increase (P < .01), and (4) decreased maximum systolic area increase (P < .001). The diagnostic power of all investigated parameters was tested by single logistic regression models. A multiple logistic regression model including solely aortic parameters yielded a sensitivity of 95% and a specificity of 100%.

CONCLUSIONS: In young patients with Marfan syndrome, a computerized image-analyzing technique revealed decreased aortic elastic properties expressed by parameters showing high diagnostic power. A multiple logistic regression model including merely aortic parameters can serve as useful predictor for Marfan syndrome.

This article has been cited by other articles:

				E. Girdauskas, T. Kuntze, M. A. Borger, T. Doenst, M. Mochalski, T. Walther, V. Falk, and F. W. Mohr Long-Term Prognosis of Type A Aortic Dissection in Non-Marfan Patients With Histologic Pattern of Cystic Medial Necrosis Ann. Thorac. Surg., March 1, 2008; 85(3): 972 - 977. [Abstract] [Full Text] [PDF]

				S. Nistri, J. Grande-Allen, M. Noale, C. Basso, P. Siviero, S. Maggi, G. Crepaldi, and G. Thiene Aortic elasticity and size in bicuspid aortic valve syndrome Eur. Heart J., February 2, 2008; 29(4): 472 - 479. [Abstract] [Full Text] [PDF]

				D. Baumgartner, C. Baumgartner, E. Schermer, G. Engl, U. Schweigmann, G. Matyas, B. Steinmann, and J. I. Stein Different patterns of aortic wall elasticity in patients with Marfan syndrome: A noninvasive follow-up study J. Thorac. Cardiovasc. Surg., October 1, 2006; 132(4): 811 - 819. [Abstract] [Full Text] [PDF]

				A. Kuhn and M. Vogt Ascending Aortic Distensibility is Impaired Before and After Surgical "Repair" of Coarctation Ann. Thorac. Surg., June 1, 2006; 81(6): 2341 - 2341. [Full Text] [PDF]

				M. Vogt, A. Kuhn, D. Baumgartner, C. Baumgartner, R. Busch, M. Kostolny, and J. Hess Impaired Elastic Properties of the Ascending Aorta in Newborns Before and Early After Successful Coarctation Repair: Proof of a Systemic Vascular Disease of the Prestenotic Arteries? Circulation, June 21, 2005; 111(24): 3269 - 3273. [Abstract] [Full Text] [PDF]