Different patterns of aortic wall elasticity in patients with Marfan syndrome: a noninvasive follow-up study.
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 beta-blocker therapy, we investigated aortic elastic properties before and during beta-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 beta-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 beta-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.
Baumgartner
Daniela
D
Department of Pediatric Cardiology, Innsbruck Medical University, Innsbruck, Austria. daniela.baumgartner@uibk.ac.at
Baumgartner
Christian
C
Schermer
Elisabeth
E
Engl
Georg
G
Schweigmann
Ulrich
U
Mátyás
Gabor
G
Steinmann
Beat
B
Stein
Jörg Ingolf
JI
D000293:Adolescent; D000319:Adrenergic beta-Antagonists / Q000494:pharmacology* / Q000627:therapeutic use; D000328:Adult; D001013:Aorta, Thoracic / Q000187:drug effects* / Q000503:physiopathology*; D001262:Atenolol / Q000494:pharmacology* / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D004548:Elasticity; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D008382:Marfan Syndrome / Q000188:drug therapy / Q000503:physiopathology*; D008875:Middle Aged
D000319:Adrenergic beta-Antagonists; D001262:Atenolol