Magnetic resonance imaging evaluation of aortic elastic properties as early expression of Marfan syndrome.
In Marfan syndrome, early identification and treatment of aortic involvement could improve prognosis, but clinical diagnosis may be difficult at a young age, before aortic dilation occurs. The aim of this study was to evaluate biomechanical aortic properties in Marfan patients and in their relatives to identify an early index of aortic involvement. A magnetic resonance imaging (MRI) morphologic and functional study of the thoracic aorta was performed in 20 Marfan patients, 15 family members, and 14 healthy volunteers as a control group. The aorta was imaged in the oblique sagittal plane by spin-echo sequence. A high-resolution gradient-echo sequence was then applied in the axial plane at the level of ascending supravalvular aorta to evaluate aortic distensibility. Aortic distensibility (mm Hg(-1) was significantly different in the three groups (ANOVA, p = 0.0001). Aortic distensibility was sensibly reduced in Marfan patients (0.0085 +/- 0.006 vs. 0.025 +/- 0.006 control group, p < 0.05). No significant correlation was found between aortic area and distensibility. Aortic distensibility was reduced also in family members (0.016 +/- 0.011 vs. 0.025 +/- 0.006 control group, p < 0.05). Among them, 4 subjects showed aortic diameters to the upper limit of the normal range, whereas the other 11 presented normal aortic diameters. Intraobserver and interobserver reproducibility for diastolic measurement was 1.2% and 0.4%, respectively, and 1.1% and 0.3%, respectively, for systolic measurement. MRI is an accurate technique in detecting abnormal aortic elastic properties in Marfan patients. Abnormal ascending aorta distensibility may constitute an index of early aortic involvement before dilation occurs.
Fattori
R
R
Institute of Radiology, University of Bologna, Italy.
Bacchi Reggiani
L
L
Pepe
G
G
Napoli
G
G
Bnà
C
C
Celletti
F
F
Lovato
L
L
Gavelli
G
G
D000293:Adolescent; D000328:Adult; D001013:Aorta, Thoracic / Q000473:pathology / Q000503:physiopathology; D001018:Aortic Diseases / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology; D001696:Biomechanical Phenomena; D002648:Child; D003971:Diastole / Q000502:physiology; D004548:Elasticity; D005260:Female; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006801:Humans; D007089:Image Enhancement; D008279:Magnetic Resonance Imaging; D008297:Male; D008382:Marfan Syndrome / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology; D012016:Reference Values; D013599:Systole / Q000502:physiology