Expert Review: Valve Disease

Predictors of Ascending Aortic Dilation in Bicuspid Aortic Valve Disease: A Five-year Prospective Study

The article focuses on the progression of aortic dilation in bicuspid aortic valve (BAV) individuals. The authors prospectively evaluate an asymptomatic cohort of BAV individuals and assess the rate of aortic growth with time. They find a mean annual rate of growth of 0.47mm/year in ascending aorta diameter (AAD), with 30% of individuals who do not show any progression at all and 15.6% who dilate >1mm/yearInterestingly, the only independent predictor of aortic growth over time is the family history of aortic valve disease.
This association is interesting in from a practical and bilateral point of view:
a) BAV has a significant familial clustering1 and meets the established criteria for disease screening in adult first-degree relatives. This is strongly supported as outlined in the AHA/ACC 2008 guidelines for the management of adults with congenital heart disease2. Therefore, once a BAV case (proband) is diagnosed, an echocardiographic screening should be offered to all the first degree relatives. This screening would ideally consider not only aortic valve, but also AAD.
b) Given the findings of Avandhani et al, aortic growth rate is higher in the familial form of BAV. Therefore, a closer AAD follow-up should be considered in BAV individuals with a family history of aortic valve disease.
However, there are some findings that could be discussed.
Firstly, the asymptomatic nature of the cohort studied limits the possible association of valve dysfunction and AAD. If valve dysfunction was not severe enough to develop symptoms, the hemodynamic stress could also be insufficient to translate into measurable ADD changes.
Secondly, the authors do not mention the prevalence and/or association with other cardiovascular malformations. It is well known the influence of aortic coarctation on aortic dimensions, its higher risk of aortic dissection, and the frequent coexistence of these two abnormalities3.
Finally, aortic evaluation has been limited to aortic root and ascending aorta, while bicuspid aortopathy is a diffuse disease that extends up to aortic arch. Therefore, the assessment of other aortic segments could have raised different results, either in the progression of aortic growth or in its association with cusp morphology or valve function.

Corresponding author from original paper
Linda A Pape
Department of Medicine, University of Massachusetts Medical School, Worcester;


1.- Kerstjens-Frederikse WS, Sarvaas GJ, Ruiter JS, et al. Left ventricular outflow tract obstruction:should cardiac screening be offered to first-degree relatives? Heart 2011;97:1228e32.
2.- Warnes CA, Williams RG, Bashore TM, et al. ACC/ AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart
Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol 2008;52:e1e121
3- Warnes CA. Bicuspid aortic valve and coarctation: two villains part of a diffuse problem. Heart. 2003;89(9):965-6.

Citation: Avadhani SA, Martin-Doyle W, Shaikh AY, Pape LA. Predictors of Ascending Aortic Dilation in Bicuspid Aortic Valve Disease: A Five-year Prospective Study. Am J Med. 2015 Published online (Jan 30).

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