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Published on: May 21, 2017
Cause of Ascending Aortic Dilatation in Patients with Bicuspid Aortic Valves: The Final Link
Francis Robicsek1, Jeko M Madjarov1, Robert F Padera2
1Department of Thoracic and Cardiovascular Surgery, Sanger Heart and Vascular Institute, Carolinas Medical Centre, Charlotte, North Carolina, United States.
Insights
Congenital bicuspid aortic valves (CBAVs) cause ascending aorta dilatation due to turbulent blood flow, not genetics, in most cases. Aortic wall structure is normal at birth when CBAV is the only defect.
Area of Science:
- Cardiovascular Medicine
- Medical Genetics
- Biomedical Engineering
Background:
- Congenital bicuspid aortic valves (CBAVs) are associated with ascending aorta dilatation.
- The etiology of this dilatation is debated, with theories including genetic factors and turbulent blood flow.
- Previous research indicated CBAVs can cause inherent morphological stenosis, even without significant pressure gradients.
Purpose of the Study:
- To investigate the cause of ascending aorta dilatation in patients with CBAVs.
- To differentiate between genetic and hemodynamic factors contributing to aortic dilatation.
- To reinforce the concept of poststenotic dilatation in CBAV patients.
Main Methods:
- Studied aortic wall structure in newborns with CBAV.
- Compared infants with CBAV as the sole anomaly versus those with additional cardiovascular defects.
Main Results:
- Aortic wall structure was normal at birth in infants with CBAV as the only cardiac anomaly.
- Aortic wall structure was abnormal at birth if additional cardiovascular anomalies were present alongside CBAV.
Conclusions:
- Ascending aorta dilatation in CBAV patients, when CBAV is the sole anomaly, is primarily due to poststenotic dilatation caused by turbulent blood flow.
- The presence of additional cardiovascular anomalies may exacerbate aortic dilatation through genetic abnormalities of the aortic wall.
Background:
This article discusses about the cause of dilatation of the ascending aorta in patients with congenitally bicuspid aortic valves (CBAVs). Some members of the profession believe that it is genetic, while others attribute it to turbulence. The author previously presented in vitro data proving that CBAVs are inherently morphological stenotic, even in the absence of measurable gradient and clinical symptoms. This article reinforces the view that the dilatation of the ascending aorta in patients with CBAV represents a "poststenotic dilatation" by demonstrating that the structure of the aortic wall is normal in infants who have CBAV as a sole cardiac anomaly.
Methods:
The aortic wall was studied in newborns who had CBAV as the sole cardiovascular anomaly and in those where CABV was associated with other inborn cardiovascular defects.
Results:
We found that in patients where CBAV is the sole cardiovascular anomaly, aortic structure is normal at birth, but abnormal if additional cardiovascular anomalies are present.
Conclusions:
Dilatation of the ascending aorta in patients, where CBAV is the only cardiovascular anomaly, is caused by turbulence (poststenotic dilatation). If additional cardiovascular anomalies are present, this process may be exaggerated by genetic abnormalities of the aortic wall.
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