• Bruno Merlanti, Benedetta De Chiara, Aldo Pietro Maggioni, Antonella Moreo, Silvana Pileggi, Gabriella Romeo, Claudio Francesco Russo, Stefania Rizzo, Luigi Martinelli, Attilio Maseri, and VAR Study Group.
• S.C. Cardiochirurgia, Department of Cardiology and Cardiovascular Surgery, Niguarda Ca' Granda Hospital, Milan, Italy.
• Int. J. Cardiol. 2015 Nov 15; 199: 180-5.

Background/ObjectivesBicuspid aortic valve (BAV) is the most common congenital heart disorder, affecting up to 2% of the population. Involvement of aortic root and ascending aorta (aneurysm or, eventually, dissection) is frequent in patients with pathologic or normal functioning BAV. Unfortunately, there are no well-known correlations between valvular and vascular diseases. In VAR protocol, with a new strategy of research, we analysemultiple aspects of BAV disease through correlation between surgical, echo, histologic and genetic findings in phenotypically homogeneous outlier cases.MethodsVAR protocol is a prospective, longitudinal, multicenter study. It observes 4 homogeneous small groups of BAV surgical patients (15 patients each): isolated aortic regurgitation, isolated ascending aortic aneurysm, aortic regurgitation associated with aortic aneurysm, isolated aortic stenosis in older patients (>60years). Echo analysis is extended to first-degree relatives and, in case of BAV, genetic test is performed. Patients and relatives are enrolled in 10 cardiac surgery/cardiologic centers throughout Italy.ConclusionsThe aim of the study is to identify predictors of favorable or unfavorable evolution of BAV in terms of valvular dysfunction and/or aortic aneurysm. Correlations between different features could help in identification of various BAV risk groups, rationalizing follow-up and treatment.Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

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