Biophysical properties of the normal-sized aorta in patients with Marfan syndrome: evaluation with MR flow mapping.
OBJECTIVE
To investigate the feasibility of magnetic resonance (MR) flow mapping in the assessment of aortic biophysical properties in patients with Marfan syndrome and to detect differences in biophysical properties in the normal-sized aorta distal to the aortic root between these patients and matched control subjects.


METHODS
Seventy-eight patients with Marfan syndrome with aortic root dilatation and 23 matched control subjects underwent MR flow mapping in four locations in the normal-sized aorta (1, ascending aorta; 2, thoracic descending aorta; 3, descending aorta at the level of the diaphragm; and 4, abdominal descending aorta). Distensibility at each location and flow wave velocity between locations were calculated.


RESULTS
Compared with the control subjects, patients with Marfan syndrome had decreased aortic distensibility at three of the four locations (levels 1, 2, and 4; P <.05) and increased flow wave velocity between all locations (P <.05) in the aorta. In patients with Marfan syndrome, flow wave velocity was also significantly increased along the entire aortic tract beyond the aortic root (from level 1 to level 4).


CONCLUSIONS
MR imaging reveals abnormal biophysical properties of the normal-sized aorta in patients with Marfan syndrome. Monitoring of these properties is relevant for evaluating disease progression and treatment options.
Groenink
M
M
Department of Cardiology, Academic Medical Center, Amsterdam, the Netherlands.
de Roos
A
A
Mulder
B J
BJ
Verbeeten
B
B
Timmermans
J
J
Zwinderman
A H
AH
Spaan
J A
JA
van der Wall
E E
EE
D000293:Adolescent; D000328:Adult; D001011:Aorta / Q000473:pathology / Q000503:physiopathology*; D001783:Blood Flow Velocity; D001794:Blood Pressure; D001830:Body Surface Area; D004548:Elasticity; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging; D008297:Male; D008382:Marfan Syndrome / Q000473:pathology / Q000503:physiopathology*; D008875:Middle Aged; D015588:Observer Variation; D015203:Reproducibility of Results