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Original Investigation
January 04, 2017

Prognostic Implications of Raphe in Bicuspid Aortic Valve Anatomy

Author Affiliations
  • 1Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands
  • 2Department of Cardiology, National University Heart Center, National University Health System, Singapore
  • 3Yong Loo Lin School of Medicine, National University of Singapore, Singapore
  • 4Department of Cardiology, Princess Alexandra Hospital, The University of Queensland, Queensland, Australia
  • 5Division of Cardiology, University of Alberta, Mazankowski Alberta Heart Institute, Alberta, Canada
  • 6University of Medicine and Pharmacy “Carol Davila”–Euroecolab, Institute of Cardiovascular Diseases “Prof Dr C. C. Iliescu,” Bucharest, Romania
  • 7Department of Cardiology, Khoo Teck Puat Hospital, Singapore
  • 8Department of Cardiology, AHEPA University Hospital, Thessaloniki, Greece
JAMA Cardiol. Published online January 4, 2017. doi:10.1001/jamacardio.2016.5228
Key Points

Question  Is there any association between bicuspid aortic valve morphologic findings (with or without raphe) and degree of valve dysfunction, presence of aortopathy, and prognosis (including need for aortic valve surgery and all-cause mortality)?

Findings  In this large international registry of 2118 patients with bicuspid aortic valves, the presence of raphe was associated with a higher prevalence of significant aortic stenosis and regurgitation and increased rates of aortic valve and aorta surgery. Although patients with bicuspid aortic valve and raphe had higher mortality rates than patients without raphe, the presence of raphe was not independently associated with increased all-cause mortality.

Meaning  The presence of raphe in bicuspid aortic valve is associated with a higher prevalence of significant valvular dysfunction and increased rates of aortic valve surgery.

Abstract

Importance  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.

Objective  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).

Design, Setting, and Participants  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.

Exposures  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.

Main Outcomes and Measures  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

Results  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.

Conclusions and Relevance  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.

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