Left Ventricular Noncompaction in Patients with Bicuspid Aortic Valve

Agarwal, Anushree; Khandheria, Bijoy K.; Paterick, J.D. Timothy E; Treiber, Shannon C; Bush, Michelle; Tajik, A. Jamil

doi:10.1016/j.echo.2013.08.003

Cited by 28 publications

(22 citation statements)

References 45 publications

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“…2019; 16(3):e85739. general population (11). Several cases illustrate an unusual association of noncompaction cardiomyopathy with morphologic and functional abnormalities of the mitral valve in the form of thickened leaflets with restricted movement (12).…”

Section: Discussionmentioning

confidence: 99%

Left Ventricular Noncompaction with Bicuspid Aortic Valve: A Case Report with a Comprehensive Cardiac MRI Approach

et al. 2019

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Noncompaction of ventricular myocardium, once thought to represent a rare cardiomyopathy, is being diagnosed with increased frequency. It is often accompanied by various congenital heart diseases. Coexisting valvular anomalies are an emerging issue that requires further investigation in patients with noncompaction cardiomyopathy, because they can increase the potential for adverse clinical events. This article describes the case of a 45-year-old male diagnosed with left ventricular noncompaction (LVNC) with a bicuspid aortic valve. Progressive deterioration of cardiac function in this patient could be explained by myocardial fibrosis and accompanying valvular anomalies on cardiac magnetic resonance imaging (MRI). The clinical implications of cardiac MRI in noncompaction cardiomyopathy are discussed based on its ability to depict both morphological and functional features. Considering the potential for coexisting congenital heart disease to declines in cardiac function, patients with LVNC should be comprehensively evaluated for concurrent cardiac anomalies and myocardial fibrosis.

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Section: Discussionmentioning

confidence: 99%

Left Ventricular Noncompaction with Bicuspid Aortic Valve: A Case Report with a Comprehensive Cardiac MRI Approach

et al. 2019

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“…We then examined other congenital malformations in the BAV vs trileaflet group. Associated left ventricular noncompaction was noted in 21 cases of BAV (3.5%) and no patients with trileaflet valves . Eleven patients in the BAV group were noted to have coarctation or coarctation repair, with none in the trileaflet group.…”

Section: Resultsmentioning

confidence: 94%

“…Male gender was more frequent in BAV group (n = 409 [69.4%]). Associated left ventricular noncompaction was noted in 21 (3.5%) cases of BAV and none in patients with trileaflet aortic valve . Concomitant aortopathy was noted in 280 cases with BAV and 16 cases without BAV.…”

Section: Resultsmentioning

confidence: 96%

Diagnostic accuracy of bicuspid aortic valve by echocardiography

et al. 2018

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Background Echocardiography is regarded as the gold standard for diagnosis of bicuspid aortic valve (BAV), yet diagnostic accuracy has been evaluated previously only in single‐center studies. We systematically evaluated the accuracy of BAV diagnosis in a large healthcare system of multiple echocardiography laboratories. Methods and Results Aurora Health Care is a multihospital, multi‐clinic system across the state of Wisconsin encompassing 33 inpatient and outpatient echocardiography laboratories with 39 cardiologist readers and 72 sonographers. As all laboratories store echocardiograms in one database, we queried all patients with “bicuspid aortic valve” diagnosis on echocardiography. Echocardiograms were classified as “BAV” or “possible BAV” based on initial reader confidence. An expert review panel categorized each as BAV, no BAV, or Indeterminate. Of the 745 cases identified, 589 (79.1%, initial reader interpretation: n = 494 “BAV,” n = 95 “possible”) were BAV. A high level of agreement (84%) was present in BAV diagnosis. There were 156 (20.9%) echocardiograms that were no BAV (8.4%) or Indeterminate (12.4%). We identified three common reasons for misdiagnosis: poor image quality (n = 70, 44.9%), leaflet calcium (n = 44, 28.2%), and oblique axis imaging (n = 33, 21.1%). A clear reason for misclassification was not elucidated in nine cases (n = 9, 5.7%). Conclusions This is the first study to evaluate BAV accuracy across a community health system with multiple echocardiography laboratories and a heterogeneous group of readers and sonographers. We establish a high degree of accuracy of echocardiography in BAV diagnosis and draw attention to common echocardiographic pitfalls that lead to BAV misclassification, highlighting opportunities for education and quality improvement.

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“…Case reports of specific CMs in BAV subjects have been sporadic; however, studies on their prevalence are limited. Agarwal et al [12] reported the incidence of LVNC in patients with BAV. In their retrospective observational study, 12 of 109 BAV patients (11.0%) were diagnosed with LVNC based on echocardiographic criteria.…”

Section: Discussionmentioning

confidence: 99%

“…Although a possible association between BAV and specific cardiomyopathies (CMs) based on common genetic traits has been proposed in several case reports [10,11], data on the prevalence of coexisting specific CMs in subjects with BAV are limited. In a previous study, an 11% incidence of left ventricular non-compaction (LVNC) in 109 patients with BAV was reported [12]. In a recent large population study, an only 0.4% prevalence of hypertrophic CM (HCM) was reported in 5,430 patients with BAV, similar to the general population [13].…”

Section: Introductionmentioning

confidence: 97%

Prevalence, Characteristics, and Clinical Significance of Concomitant Cardiomyopathies in Subjects with Bicuspid Aortic Valve

Jeong

Shim

Kim

et al. 2018

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24Background: In this study, the prevalence, characteristics, and clinical significance of 25 concomitant specific cardiomyopathies were investigated in subjects with bicuspid aortic 26 valve (BAV). 27 Methods: We retrospectively evaluated 1,186 adults with BAV (850 males, mean age 56 ± 28 14 years). Left ventricular non-compaction, hypertrophic cardiomyopathy, and idiopathic 29 dilated cardiomyopathy were diagnosed when patients fulfilled current clinical and 30 echocardiographic criteria. Clinical and echocardiographic characteristics including 31 comorbidities, heart failure presentation, BAV morphology, function, and aorta phenotypes in 32 BAV subjects with or without specific cardiomyopathies were compared. 33 Results: Overall, 67 subjects (5.6 %) had concomitant cardiomyopathies: 40 (3.4%) patients 34 with left ventricular non-compaction, 17 (1.4%) with hypertrophic cardiomyopathy, and 10 35 (0.8%) with dilated cardiomyopathy. BAV subjects with hypertrophic cardiomyopathy had a 36higher prevalence of diabetes mellitus, heart failure with preserved ejection fraction, and 37 tended to have type 0 phenotype, while BAV subjects with dilated cardiomyopathy showed a 38 higher prevalence of chronic kidney disease and heart failure with reduced ejection fraction. 39BAV subjects with left ventricular non-compaction were significantly younger, 40 predominantly male, and had greater BAV dysfunction and a higher prevalence of normal 41 aorta shape. In multiple regression analysis, presence of cardiomyopathy was independently 42 associated with heart failure (odds ratio 2.866, 95% confidential interval 1.652-4.974, p < 43 0.001) even after controlling confounding factors. 44 Conclusion: Concomitant cardiomyopathies were observed in 5.6% of subjects with BAV. A 45 few clinical and echocardiographic characteristics including comorbidities, heart failure 46 presentation, BAV morphology, function, and presence of aortopathy were found. The 3 47 presence of cardiomyopathy was independently associated with heart failure. 48 Keywords: Bicuspid aortic valve, left ventricular non-compaction, hypertrophic 49 cardiomyopathy, dilated cardiomyopathy 50 51 Abbreviations 52 BAV = bicuspid aortic valve 53 LVNC = left ventricular non-compaction 54 CM = cardiomyopathy 55 HCM = hypertrophic cardiomyopathy 56 DCM = dilated cardiomyopathy 57 LVEF = left ventricular ejection fraction 58 OR = odds ratio 59 CI = confidence interval 60 61 62 63 64 65 66 67 4 68 131 failure with preserved ejection fraction (LVEF ≥ 50%) and heart failure with reduced ejection 132 fraction (LVEF < 50%). 133 Continuous variables were expressed as mean ± standard deviation (SD). Categorical 134 variables were expressed as number (percentage). Comparisons between the groups were 135 performed using standard λ 2 tests for categorical variables and paired t-tests for continuous 136 variables. Univariate and multivariate logistic regression analyses were performed. All 137 statistical analyses were performed using SPSS Statistics, software version 23.0 (IBM, 138 Armonk,...

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Left Ventricular Noncompaction in Patients with Bicuspid Aortic Valve

Cited by 28 publications

References 45 publications

Left Ventricular Noncompaction with Bicuspid Aortic Valve: A Case Report with a Comprehensive Cardiac MRI Approach

Left Ventricular Noncompaction with Bicuspid Aortic Valve: A Case Report with a Comprehensive Cardiac MRI Approach

Diagnostic accuracy of bicuspid aortic valve by echocardiography

Prevalence, Characteristics, and Clinical Significance of Concomitant Cardiomyopathies in Subjects with Bicuspid Aortic Valve

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