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Left ventricular noncompaction in patients with bicuspid aortic valve
Anushree Agarwal1, Bijoy K Khandheria, Timothy E Paterick
1Aurora Cardiovascular Services, Aurora Sinai/Aurora St. Luke's Medical Centers, University of Wisconsin School of Medicine and Public Health, Milwaukee, Wisconsin.
Insights
Bicuspid aortic valve (BAV) and left ventricular noncompaction (LVNC) co-occur in 11% of BAV patients. This study characterized the clinical and imaging features of this combined cardiac anomaly in a retrospective echocardiographic analysis.
Area of Science:
- Cardiology
- Medical Imaging
- Genetics
Background:
- Left ventricular noncompaction (LVNC) is typically linked to complex congenital heart defects.
- The association between LVNC and bicuspid aortic valve (BAV), a common anomaly, is poorly documented.
Purpose of the Study:
- To determine the incidence of LVNC in patients with BAV.
- To describe the clinical and echocardiographic characteristics of patients with both BAV and LVNC.
Main Methods:
- Retrospective analysis of an echocardiography database from July 2011 to March 2013.
- Identification of 109 patients with BAV and subsequent evaluation for concomitant LVNC.
Main Results:
- 11.0% (12/109) of BAV patients met criteria for LVNC.
- The average age at diagnosis was 33 years, with a male predominance (9/12).
- Common findings included BAV with right/left cusp fusion, mild/moderate aortic valve dysfunction, and apical LVNC.
Conclusions:
- BAV and LVNC coexist in approximately 11% of BAV patients.
- Further research is required to elucidate the underlying genetic and pathophysiological mechanisms of this association.
Background:
Left ventricular noncompaction (LVNC) is commonly associated with complex congenital anomalies. The association of LVNC with less complex but more frequent anomalies, such as bicuspid aortic valve (BAV), is not well described in the literature. The aims of this study were to (1) determine the incidence of association of LVNC with the most common congenital anomaly, BAV, in an echocardiographic database and (2) describe clinical and imaging characteristics of these patients.
Methods:
An echocardiography database was retrospectively interrogated to identify 109 patients who fulfilled the echocardiographic criteria for BAV from July 1, 2011, to March 31, 2013. Echocardiograms were carefully evaluated to identify patients with concomitant LVNC.
Results:
Twelve patients (11.0%) with BAV fulfilled the criteria for LVNC. The mean age at diagnosis was 33 ± 16.9 years; nine of 12 were men. Eight patients (66.7%) had symptoms during initial presentation. The most common BAV morphology was fusion of the right and left coronary cusps. Nine patients had mild or moderate aortic valve dysfunction (aortic regurgitation and/or stenosis), and eight had associated aortopathy. LVNC was located at the apex in all patients except one. Mean systolic global longitudinal strain was -16.9 ± 2.7%.
Conclusions:
In this series of patients, concomitant BAV and LVNC were observed in 11% of a BAV population. Further studies are needed to understand the genetic and pathophysiologic basis of this association.
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