Determinants of rapid progression of aortic root dilatation and complications in Marfan syndrome.
BACKGROUND
Progressive aortic dilatation has prognostic significance in the Marfan syndrome.


METHODS
To identify which patients were at high risk of rapid progression, we echocardiographically studied 43 patients (age 22 +/- 14 years) with the mean follow-up period of 5.2 +/- 3.2 years. Aortic diameters, left ventricular (LV) size, fractional shortening, and the severity of aortic and mitral regurgitation were assessed. Transmitral peak early and atrial flow velocities, their ratio and the deceleration time of peak early velocity were also obtained.


RESULTS
Mean annual increases of aortic diameters were 0.4 +/- 0.3 mm at the annulus, 1.5 +/- 1.3 mm at the sinuses of Valsalva, 0.7 +/- 0.6 mm at the supraaortic ridge and 0.4 +/- 0.4 mm at the proximal ascending aorta. Patients were divided into 2 groups according to the aortic growth rate at the sinuses of Valsalva level: rapid (R, >3% per year, 15 patients) or slow (S, < or =3% per year, 28 patients) progression groups. Measured variables did not show significant differences between the 2 groups except older age, higher blood pressure and more severe aortic regurgitation in group R. Multiple regression analysis identified prolonged deceleration time as the most important variable predicting aortic complications. Aortic dissection occurred more frequently in group R (7 patients, 47%) than in group S (0%, P < 0.001).


CONCLUSIONS
Marfan patients at older age, with higher blood pressure, and with significant aortic regurgitation were at high risk of progression of aortic dilatation, with the most remarkable increase at the sinuses of Valsalva. Prolonged deceleration time may relate to an increased risk for aortic complications.
Lazarevic
Aleksandar M
AM
Department of Cardiology, National Cardiovascular Center, Suita, Osaka, Japan.
Nakatani
Satoshi
S
Okita
Yutaka
Y
Marinkovic
Jelena
J
Takeda
Yutaka
Y
Hirooka
Keiji
K
Matsuo
Hiroshi
H
Kitamura
Soichiro
S
Yamagishi
Masakazu
M
Miyatake
Kunio
K
D000293:Adolescent; D000328:Adult; D001018:Aortic Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D004108:Dilatation, Pathologic; D018450:Disease Progression; D004452:Echocardiography; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology*; D008875:Middle Aged; D012044:Regression Analysis; D012189:Retrospective Studies