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  • Kong, William K F; Delgado, Victoria; Poh, Kian Keong; Regeer, Madelien V; Ng, Arnold C T; McCormack, Louise; Yeo, Tiong Cheng; Shanks, Miriam; Parent, Sarah; Enache, Roxana; Popescu, Bogdan A; Liang, Michael; Yip, James W; Ma, Lawrence C W; Kamperidis, Vasileios; van Rosendael, Philippe J; van der Velde, Enno T; Ajmone Marsan, Nina; Bax, Jeroen J

    JAMA cardiology, 03/2017, Letnik: 2, Številka: 3
    Journal Article

    Little is known about the association between bicuspid aortic valve (BAV) morphologic findings and the degree of valvular dysfunction, presence of aortopathy, and complications, including aortic valve surgery, aortic dissection, and all-cause mortality. To investigate the association between BAV morphologic findings (raphe vs nonraphe) and the degree of valve dysfunction, presence of aortopathy, and prognosis (including need for aortic valve surgery, aortic dissection, and all-cause mortality). In this large international multicenter registry of patients with BAV treated at tertiary referral centers, 2118 patients with BAV were evaluated. Patients referred for echocardiography from June 1, 1991, through November 31, 2015, were included in the study. Clinical and echocardiographic data were analyzed retrospectively. The morphologic BAV findings were categorized according to the Sievers and Schmidtke classification. Aortic valve function was divided into normal, regurgitation, or stenosis. Patterns of BAV aortopathy included the following: type 1, dilation of the ascending aorta and aortic root; type 2, isolated dilation of the ascending aorta; and type 3, isolated dilation of the sinus of Valsalva and/or sinotubular junction. Association between the presence and location of raphe and the risk of significant (moderate and severe) aortic valve dysfunction and aortic dilation and/or dissection. Of the 2118 patients (mean SD age, 47 18 years; 1525 72.0% male), 1881 (88.8%) had BAV with fusion raphe, whereas 237 (11.2%) had BAV without raphe. Bicuspid aortic valves with raphe had a significantly higher prevalence of valve dysfunction, with a significantly higher frequency of aortic regurgitation (622 33.1% vs 57 24.1%, P < .001) and aortic stenosis (728 38.7% vs 51 21.5%, P < .001). Furthermore, aortic valve replacement event rates were significantly higher among patients with BAV with raphe (364 19.9% at 1 year, 393 21.4% at 2 years, and 447 24.4% at 5 years) vs patients without raphe (30 14.0% at 1 year, 32 15.0% at 2 years, and 40 18.0% at 5 years) (P = .02). In addition, the all-cause mortality event rates were significantly higher among patients with BAV with raphe (77 5.1% at 1 year, 87 6.2% at 2 years, and 110 9.5% at 5 years) vs patients without raphe (2 1.8% at 1 year, 3 3.0% at 2 years, and 5 4.4% at 5 years) (P = .03). However, on multivariable analysis, the presence of raphe was not significantly associated with all-cause mortality. In this large multicenter, international BAV registry, the presence of raphe was associated with a higher prevalence of significant aortic stenosis and regurgitation. The presence of raphe was also associated with increased rates of aortic valve and aortic surgery. Although patients with BAV and raphe had higher mortality rates than patients without, the presence of a raphe was not independently associated with increased all-cause mortality.