Restricted cusp motion in right-left type of bicuspid aortic valves: a new risk marker for aortopathy.

OBJECTIVE: Bicuspid aortic valve disease is heterogeneous with respect to valve morphology and aortopathy risk. This study searched for early imaging predictors of aortopathy in patients with a bicuspid aortic valve with right-left coronary cusp fusion, the most common morphotype. METHODS: Time-resolved magnetic resonance imaging was performed in 36 subjects with nonstenotic, nonregurgitant bicuspid aortic valves and nondilated aortas and in 10 healthy controls with tricuspid aortic valves. Sinus dimensions (diameter, width, and height), ascending tract diameters, and wall strain were measured for each sinus/leaflet unit and corresponding ascending tract area to account for asymmetries. A novel parameter, "cusp opening angle," measured the degree of valve leaflet alignment to outflow axis in systole, quantifying cusp motility. Phase-contrast magnetic resonance imaging and computational fluid dynamic models assessed flow patterns. Aortic growth rate was estimated over a follow-up period ranging from 9 to 84 months. RESULTS: The expected restriction of bicuspid aortic valve opening (conjoint cusp opening angle, 62°±5° vs 76°±3° for nonfused leaflet and 75°±3° for tricuspid aortic valve cusps; P<.001) was confirmed, and the introduced parameter reproducibly quantified this phenomenon. Phase-contrast magnetic resonance imaging demonstrated systolic flow deflection toward the right, affecting the right anterolateral ascending wall. Computational models confirmed that restricted cusp motion alone is sufficient to cause the observed flow pattern. Ascending tract wall strain was not circumferentially homogeneous in bicuspid aortic valves. In multivariable analyses, the conjoint cusp opening angle independently predicted ascending aorta diameters and growth rate (P<.001). CONCLUSIONS: In the bicuspid aortic valve commonly defined as normofunctional by echocardiographic criteria, restricted systolic conjoint cusp motion causes flow deflection. The novel measurement introduced can quantify restricted cusp opening, possibly assuming prognostic importance.