Clinical Investigation
Congenital Heart Disease
Left Ventricular Noncompaction in Patients with Bicuspid Aortic Valve

https://doi.org/10.1016/j.echo.2013.08.003Get rights and content

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.

Section snippets

Patients

Our echocardiography database was retrospectively interrogated for the diagnosis of BAV and LVNC from July 1, 2011, to March 31, 2013. We identified 109 patients with BAV within our database. We also interrogated our echocardiographic database for calendar year 2012 to retrospectively analyze the annual prevalence of LVNC among patients who were referred to our echocardiographic laboratory. Approval was obtained from our institutional review board.

Diagnostic Criteria for LVNC

Several echocardiographic and cardiac magnetic

Study Population

In 2012, a total of 17,016 patients were referred to our echocardiographic laboratory for evaluation of multiple and varied cardiac disorders. Among these, 59 patients were diagnosed with LVNC, so the annual prevalence of LVNC at our institution was estimated at approximately 0.3% (three in 1,000) among patients referred for echocardiography. Of the 109 patients with BAV identified within our echocardiographic database from July 2011 to March 2013, 12 (11.0%) fulfilled the criteria for LVNC.

Discussion

At our institution, where the annual prevalence of LVNC among patients referred to our echocardiographic laboratory is 0.3%, we report an 11% incidence of LVNC in our BAV population. This series highlights an important association between two apparently diverse entities and raises questions regarding their pathophysiologic relationship.

In the 1980s, Jenni et al.12 included an absence of coexisting cardiac anomalies as one of the diagnostic criteria of LVNC. We now know that LVNC can occur

Conclusions

Concomitant BAV and LVNC is a recently defined association that needs further investigation. A major query is whether these entities interact to hasten the potential for adverse clinical events. Cardiac morphology, physiology, and genetics are the variables needed to better define whether concomitant BAV and LVNC has increased risk for adverse clinical events.

Acknowledgments

We gratefully acknowledge the editorial assistance of Joe Grundle and Katie Klein and the figure preparation of Brian Miller and Brian Schurrer, all of Aurora Cardiovascular Services. We would also like to thank Matt Umland, RDCS, and Christopher Kramer, RDCS, for their technical assistance.

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