Cited 9 times since 2016 (1.9 per year) source: Scopus Netherlands heart journal : monthly journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation, Volume 24, Issue 2, 01 February 2016, Pages 127-133 The extent of the raphe in bicuspid aortic valves is associated with aortic regurgitation and aortic root dilatation. Koenraadt WM, Grewal N, Gaidoukevitch OY, DeRuiter MC, Gittenberger-de Groot AC, Bartelings MM, Holman ER, Klautz RJ, Schalij MJ, Jongbloed MR


The clinical course of bicuspid aortic valves (BAVs) is variable. Data on predictors of aortopathy and valvular dysfunction mainly focus on valve morphology.


To determine whether the presence and extent of the raphe (fusion site of valve leaflets) is associated with the degree of aortopathy and valvular dysfunction in patients with isolated BAV and associated aortic coarctation (CoA).


Valve morphology and aortic dimensions of 255 BAV patients were evaluated retrospectively by echocardiography.


BAVs with a complete raphe had a significantly higher prevalence of valve dysfunction (especially aortic regurgitation) than BAVs with incomplete raphes (82.9 vs. 66.7 %, p = 0.01). Type 1A BAVs (fusion of right and left coronary leaflets) and complete raphe had larger aortic sinus diameters compared with the rest of the population (37.74 vs. 36.01, p = 0.031). Patients with CoA and type 1A BAV had significantly less valve regurgitation (13.6 vs. 55.8 %, p < 0.001) and smaller diameters of the ascending aorta (33.7 vs. 37.8 mm, p < 0.001) and aortic arch (25.8 vs. 30.2 mm, p < 0.001) than patients with isolated BAV.


Type 1A BAV with complete raphe is associated with more aortic regurgitation and root dilatation. The majority of CoA patients have incomplete raphes, associated with smaller aortic root diameters and less valve regurgitation.

Keywords: Echocardiography, Heart defects, congenital, Aortic Diseases, Valvular Heart Disease

Neth Heart J. 2016 Feb;24(2):127-133