Journal of the American Society of Echocardiography
Clinical InvestigationCongenital Heart DiseaseLeft Ventricular Noncompaction in Patients with Bicuspid Aortic Valve
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.
References (52)
- et al.
Bicuspid aortic valve is heritable
J Am Coll Cardiol
(2004) - et al.
A new disease: bicuspid aortic valve aortopathy syndrome
Am J Med
(2012) - et al.
Identification of a rare congenital anomaly of the myocardium by two-dimensional echocardiography: persistence of isolated myocardial sinusoids
Am J Cardiol
(1984) - et al.
Left ventricular non-compaction: genetic heterogeneity, diagnosis and clinical course
Int J Cardiol
(2010) - et al.
Left ventricular noncompaction: a pathological study of 14 cases
Hum Pathol
(2005) - et al.
Noncompacted myocardium in Ebstein’s anomaly: initial description in three patients
J Am Soc Echocardiogr
(2004) - et al.
Ebstein's anomaly with left ventricular noncompaction and bicuspid aortic valve
J Am Coll Cardiol
(2010) - et al.
Aortic valve replacement for aortic regurgitation in a patient with left ventricular noncompaction
Ann Thorac Surg
(2009) - et al.
Left ventricular noncompaction: a 25-year odyssey
J Am Soc Echocardiogr
(2012) - et al.
Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging
J Am Coll Cardiol
(2005)
Left ventricular hypertrabeculation/noncompaction
J Am Soc Echocardiogr
Recommendations for chamber quantification: a report from the American Society of Echocardiography's Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardiology
J Am Soc Echocardiogr
Recommendations for evaluation of the severity of native valvular regurgitation with two-dimensional and Doppler echocardiography
J Am Soc Echocardiogr
Recommendations for the evaluation of left ventricular diastolic function by echocardiography
J Am Soc Echocardiogr
Two-dimensional strain-a novel software for real-time quantitative echocardiographic assessment of myocardial function
J Am Soc Echocardiogr
Reduced left ventricular compacta thickness: a novel echocardiographic criterion for non-compaction cardiomyopathy
J Am Soc Echocardiogr
Novel NOTCH1 mutations in patients with bicuspid aortic valve disease and thoracic aortic aneurysms
J Thorac Cardiovasc Surg
Aortic and left ventricular remodeling in patients with bicuspid aortic valve without significant valvular dysfunction: a prospective study
Int J Cardiol
Clinical and pathophysiological implications of a bicuspid aortic valve
Circulation
The bicuspid aortic valve—an autopsy study
Indian J Pathol Microbiol
Stenotic bicuspid aortic valve associated with a ventricular septal defect in an adult presenting with congestive heart failure: a rare observation
Eur Heart J
Novel gene mutations in patients with left ventricular noncompaction or Barth syndrome
Circulation
Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity?
Eur Heart J
Persisting myocardial sinusoids of both ventricles as an isolated anomaly: echocardiographic, angiographic, and pathologic anatomical findings
Cardiovasc Intervent Radiol
Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy
Heart
Noncompaction of the myocardium associated with Roifman syndrome
Cardiol Young
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Hypertrabeculation; a phenotype with Heterogeneous etiology
2021, Progress in Cardiovascular DiseasesCitation Excerpt :The most frequent congenital defects reported were as follows: LV outflow tract abnormalities (46%), Ebstein anomaly (25%), and tetralogy of Fallot (8%).52 However, many other congenital heart disease patients have manifested LVHT.52,55–57 LVHT has also been associated with numerous systemic NMD such as mitochondrial disorders, muscular dystrophies, Danon disease, Emery-Dreifuss, Charcot-Marie-Tooth type1, metabolic myopathies, and Barth syndrome.30,36,58–60
Regression of left ventricular hypertrabeculation is associated with improvement in systolic function and favorable prognosis in adult patients with non-ischemic cardiomyopathy
2016, Journal of CardiologyCitation Excerpt :Jenni's echocardiographic criterion, namely a NC/C ratio of >2 obtained at end-systole in a parasternal short-axis view, is the most commonly used criterion for the detection of LVNC [10,29–31]. It has previously been validated against dilated cardiomyopathy, hypertensive heart disease, and valvular heart disease and showed good sensitivity and specificity [32,33]. For this reason, we used Jenni's echocardiographic criteria for the selection of the patients in our study.
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2020, Progress in Cardiovascular DiseasesCitation Excerpt :LVNC is found in conjunction with congenital heart defects in more than 10% of cases, predominately LVOT abnormalities including BAV.49 In addition, LVNC may be more common in BAV patients than in the general population,50 and BAV patients with LVNC may develop earlier onset aortic valve disease or TAA with a more malignant course requiring surgical intervention.50,51 There is limited evidence that the same genetic mutations that cause isolated LVNC can also cause BAV, but the genetic causes of most LVNC cases remain unknown.
Left ventricular non-compaction in a child with bicuspid aortic valve and aortic coarctation
2019, Cardiology in the YoungLeft ventricular non-compaction associated with bicuspid aortic valve and aortic coarctation
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