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Heartbeat: phenotypic heterogeneity of bicuspid aortic valve disease
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  1. Catherine M Otto
  1. Correspondence to Professor Catherine M Otto, Division of Cardiology, University of Washington, Seattle, WA 98195, USA; cmotto{at}uw.edu

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Bicuspid aortic valve (BAV) disease is common, affecting 1%–2% of the entire population, with nearly all BAV patients eventually requiring aortic valve replacement, often with concurrent aortic surgery for dilation of the sinuses or ascending aorta. Although echocardiography allows easy diagnosis of the presence of BAV disease early in life, we are unable to predict disease progression or aortic dilation in an individual patient and we have no effective therapies to preserve normal valve function.

In this issue of Heart, Evangelista and colleagues1 sought to identify phenotypic predictors of valve dysfunction and aortic root dilation in a series of 802 consecutive adults diagnosed with BAV at eight tertiary care hospitals. As in previous studies, BAV leaflet morphology characterised by fusion of the right and left (RL) coronary cusps was most common (73%), followed by fusion of the right and non-coronary cusps (24%). Aortic regurgitation was associated with male sex and leaflet prolapse. Aortic stenosis was associated with RL valve morphology, the presence of a raphe (seen in 82%), age, dyslipidaemia and smoking. Dilation of the ascending aorta was not associated with any specific valve morphology whereas aortic sinus dilation was associated with RL leaflet morphology, male sex, and aortic regurgitation. (figure 1)

Figure 1

Aortic diameters according to age. Graph representing mean aortic diameters at the level of the aortic root, ascending aorta and aortic arch in …

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