Background: The aim of this study was to evaluate the natural progression of aortic dilatation and its association with aortic valve stenosis (AoS) in patients with bicuspid aortic valve (BAV). Methods: Prospective study of aorta dilatation in patients with BAV and AoS using cardiac magnetic resonance (CMR). Aortic root, ascending aorta, aortic peak velocity, left ventricular systolic and diastolic function and mass were assessed at baseline and at 3-year follow-up. Results: Of the 33 enrolled patients, 5 needed surgery, while 28 patients (17 male; mean age: 31 ± 8 years) completed the study. Aortic diameters significantly increased at the aortic annulus, sinus of Valsalva and tubular ascending aorta levels (P < 0.050). The number of patients with dilated tubular ascending aortas increased from 32 % to 43 %. No significant increase in sino-tubular junction diameter was observed. Aortic peak velocity, ejection fraction and myocardial mass significantly increased while the early/late filling ratio significantly decreased at follow-up (P < 0.050). The progression rate of the ascending aorta diameter correlated weakly with the aortic peak velocity at baseline (R2= 0.16, P = 0.040). Conclusion: BAV patients with AoS showed a progressive increase of aortic diameters with maximal expression at the level of the tubular ascending aorta. The progression of aortic dilatation correlated weakly with the severity of AoS. Key Points: • Bicuspid aortic valve (BAV) is the most common congenital heart defect. • BAV patients have an increased risk of developing aortic valve stenosis (AoS). • BAV patients have an increased risk of developing thoracic aorta dilatation. • The severity of aortic stenosis is correlated to the progression of aortic dilatation. • Cardiac magnetic resonance can rapidly assess patients with a bicuspid aortic valve.

adult, aorta, aorta root, aorta sinus, aorta valve stenosis, aortic peak velocity, artery dilatation, article, ascending aorta, bicuspid aortic valve, bicuspid aortic valve stenosis, blood vessel diameter, cardiovascular magnetic resonance, cardiovascular parameters, clinical article, controlled study, disease association, disease severity, female, follow up, heart ejection fraction, heart left ventricle contraction, heart left ventricle function, heart left ventricle mass, heart output, hemodynamic parameters, human, male, priority journal, prospective study, randomized controlled trial, single blind procedure, sinotubular junction,
European Radiology: journal of the European Congress of Radiology
Erasmus MC: University Medical Center Rotterdam

Rossi, A.G, van der Linde, D, Yap, S.C, Lapinskas, T, Kirschbaum, S.W.M, Springeling, T, … Roos-Hesselink, J.W. (2013). Ascending aorta dilatation in patients with bicuspid aortic valve stenosis: A prospective CMR study. European Radiology: journal of the European Congress of Radiology, 23(3), 642–649. doi:10.1007/s00330-012-2651-7