Abstract 18425: Total Aortic Volume as a Predictor of Aortic Complications in Marfan Syndrome
Background: Mortality and morbidity in Marfan syndrome, an autosomal dominant systemic connective tissue disorder, is mainly determined by aortic complications (development of aortic aneurysm and subsequent dissection). Dissection of the descending aorta usually occurs at relatively normal aortic diameters and is therefore difficult to predict. The aim of this study was to assess total aortic volume as a predictor for aortic complications in Marfan syndrome.
Methods: In 96 adult MFS patients a Gadolinium enhanced 3D MRI of the aorta was performed. Total aortic volume (aortic valve - aortic bifurcation) measurement was performed with 3D vessel analysis software (3mensio vascular, 3mensio medical imaging BV, Bilthoven, the Netherlands). Aortic complications (aortic dissection and aortic surgery) during 3 years follow-up were collected from patients’ medical charts.
Results: In the Marfan patients (68 % male), 22% with a history of prophylactic aortic root repair, mean age 36 ± 12 years , mean body surface area (BSA) 2.06 ± 0.24 m2) a mean aortic volume of 114 ± 25ml/m2 was calculated. Aortic complications during 3 year follow-up occurred in 17 Patients. Prophylactic aortic root replacement was performed in 13 patients (14%). Descending aortic dissection occurred in 4 patients, all after previous aortic root replacement and without aortic dilatation in the dissected parts. Mean aortic volume in patients who underwent aortic surgery during the follow-up was significantly higher as compared to the uncomplicated patients (127 ± 22 ml/m2 vs 107 ± 23 ml/m2 p = 0.006). Mean aortic volume in patients with descending aortic dissection was significantly higher as compared to the uncomplicated patients (138 ± 32 ml/m2 vs 107 ± 23 ml/m2 p = 0.012). Regression analysis revealed that total aortic volume corrected for BSA may serve as a predictor for aortic root replacement and as a predictor for descending aortic dissection (OR 1.034 95 % CI 1.008 - 1.061 p = 0,011 and OR 1.042 95 % CI 1.004 - 1. 081 p= 0.028 respectively).
Conclusion: Aortic volumes may predict aortic events in Marfan syndrome.
- © 2012 by American Heart Association, Inc.