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J Thorac Cardiovasc Surg 2006;132:811-819
© 2006 The American Association for Thoracic Surgery

Surgery for Acquired Cardiovascular Disease

Different patterns of aortic wall elasticity in patients with Marfan syndrome: A noninvasive follow-up study

^a Department of Pediatric Cardiology, Innsbruck Medical University, Innsbruck, Austria
^b Research Group for Clinical Bioinformatics, Institute of Biomedical Engineering, University for Health Sciences, Medical Informatics and Technology Hall, Tyrol, Austria
^c Division of Metabolism and Molecular Pediatrics, University Children's Hospital, Zurich, Switzerland
^d Division of Medical Molecular Genetics and Gene Diagnostics, Institute of Medical Genetics, University of Zurich, Schwerzenbach, Switzerland.

Received for publication April 22, 2006; revisions received June 20, 2006; accepted for publication July 7, 2006.

^_* Address for reprints: Daniela Baumgartner, MD, Department of Pediatric Cardiology, Innsbruck Medical University, Anichstrasse 35, A 6020 Innsbruck, Austria. (Email: daniela.baumgartner{at}uibk.ac.at).

Objective: Aortic complications determine the life expectancy of most patients with Marfan syndrome. To find out whether there is heterogenous aortic involvement among patients and, if there is, to characterize aortic patterns and response to long-term ß-blocker therapy, we investigated aortic elastic properties before and during ß-blocker treatment.

Methods: In 46 patients with Marfan syndrome (age, 17.4 ± 11.1 years) and 46 healthy control subjects, ascending and descending aortic elastic parameters were determined noninvasively before and after 39 ± 16 months of ß-blocker treatment with atenolol.

Results: Aortic diameters and distensibility distinguished Marfan patients and controls with a sensitivity of 85% and a specificity of 87%. Cluster analysis revealed 4 patterns of aortic phenotypic expression: (1) reduced ascending aortic elasticity (46% of patients), (2) diminished ascending and descending aortic elasticity (17%), (3) minimal alterations of ascending and descending aortic elasticity (20%), and (4) reduced descending aortic elasticity (17%). During follow-up, aortic elastic properties improved in 21 (70%) of 30 patients and deteriorated in 9 (30%) irrespective of ß-blocker dosage. Improvement was observed in 100% of patients (n = 7; age, 5.3 ± 4.2 years) with end-diastolic aortic root diameters between 20 and 30 mm and in 61% of patients (14/23; age, 20.5 ± 10.0 years) with root diameters between 30 and 52 mm.

Conclusions: Aortic elastic parameters distinguish between patients with Marfan syndrome and healthy controls and show the pattern of regional aortic involvement. Improvement or deterioration during follow-up can influence therapeutic decisions to prevent aortic dissection and rupture. Young age, small root diameter, and high distensibility are favorable prognostic factors.

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