Left Ventricular Noncompaction

Sarma, Radha J.; Chana, Amar; Elkayam, Uri

doi:10.1016/j.pcad.2009.11.001

Cited by 96 publications

(74 citation statements)

References 104 publications

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“…Interethnic differences were best measured in the entire cohort and attenuated to some extent in the reference groups where subgrouping led to under representation of specific ethnic or racial subgroups. The association between African background and increased trabeculation has been previously reported (2,6,19,20), but fractal analysis additionally shows a positive and negative association, respectively, between Hispanic and Chinese American participants with trabeculation.…”

Section: Regional Patterns and Reference Values For LV Trabeculationsupporting

confidence: 56%

Fractal Analysis of Myocardial Trabeculations in 2547 Study Participants: Multi-Ethnic Study of Atherosclerosis

Captur¹,

Zemrak²,

Muthurangu³

et al. 2015

Radiology

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).q RSNA, 2015 Purpose:To quantitatively determine the population variation and relationship of left ventricular (LV) trabeculation to LV function, structure, and clinical variables. Materials andMethods:This HIPAA-compliant multicenter study was approved by institutional review boards of participating centers. Results:Included were 2547 participants (mean age, 68.7 years 6 9.1 [standard deviation]; 1211 men). FD MaxApical are in arbitrary units. FD MaxApical reference ranges for BMI 30 kg/m 2 or greater (n = 163), 25 kg/m 2 or greater to less than 30 kg/m 2 (n = 206), and less than 25 kg/m 2 (n = 235) were 1.203 6 0.06 (95% confidence interval: 1.194, 1.212), 1.194 6 0.06 (95% confidence interval: 1.186, 1.202), and 1.169 6 0.05 (95% confidence interval: 1.162, 1.176), respectively. In the entire cohort, adjusted for anthropometrics, trabeculation was higher in African American participants (standardized b [sb] = 0.09; P  .001) and Hispanic participants (sb = 0.05; P = .013) compared with white participants and was also higher in African American participants compared with Chinese American participants (sb = 0.08; P = .01), and this persisted after adjustment for hypertension and LV size. Hypertension (sb = 0.07; P , .001), LV mass (sb = 0.22; P , .001), and wall thickness (sb = 0.27; P , .001) were positively associated with FD MaxApical even after adjustment. In the group with BMIs less than 25 kg/ m 2 , Chinese American participants had less trabeculation than white participants (sb = 20.15; P = .032). Conclusion:Fractal analysis of cardiac MR imaging data measures endocardial complexity, which helps to differentiate normal from abnormal trabecular patterns in healthy versus diseased hearts. Trabeculation is influenced by race and/or ethnicity and, more importantly, by cardiac loading conditions and comorbidities. Clinicians who interpret cine MR imaging data should expect slightly less endocardial complexity in Chinese American patients and more in African American patients, Hispanic patients, hypertensive patients, and those with hypertrophy.q RSNA, 2015

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Section: Regional Patterns and Reference Values For LV Trabeculationsupporting

confidence: 56%

Fractal Analysis of Myocardial Trabeculations in 2547 Study Participants: Multi-Ethnic Study of Atherosclerosis

Captur¹,

Zemrak²,

Muthurangu³

et al. 2015

Radiology

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“…133,134 LVNC can be a mild disease revealed by a depressed systolic function 135 or can evolve into a serious condition with complications including systemic embolism, malignant arrhythmias, heart failure, and sudden death. 136 LVNC is generally transmitted as an autosomal dominant trait, but the underlying molecular mechanisms are still poorly understood. 134 Conditional inactivation in the embryonic mouse myocardium of the Mib1 gene, encoding the E3 ubiquitin ligase Mib1 essential for Notch ligand signaling 14 ( Figure 1), causes a phenotype strongly reminiscent of LVNC.…”

Section: Noncompactionmentioning

confidence: 99%

Endocardial Notch Signaling in Cardiac Development and Disease

Luxán

D’Amato

MacGrogan

et al. 2016

Circulation Research

198

144

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Abstract:The Notch signaling pathway is an ancient and highly conserved signaling pathway that controls cell fate specification and tissue patterning in the embryo and in the adult. Region-specific endocardial Notch activity regulates heart morphogenesis through the interaction with multiple myocardial-, epicardial-, and neural crestderived signals. Mutations in NOTCH signaling elements cause congenital heart disease in humans and mice, demonstrating its essential role in cardiac development. Studies in model systems have provided mechanistic understanding of Notch function in cardiac development, congenital heart disease, and heart regeneration. Notch patterns the embryonic endocardium into prospective territories for valve and chamber formation, and later regulates the signaling processes leading to outflow tract and valve morphogenesis and ventricular trabeculae compaction. Alterations in NOTCH signaling in the endocardium result in congenital structural malformations that can lead to disease in the neonate and adult heart. (Circ Res. 2016;118:e1-e18.

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“…Most cases are characterized by such symptoms as heart failure accompanied by dyspnoea during incrementally lowered exertion as well as orthopnoea and oedema in the lower extremities. There is a greater susceptibility to ventricular and atrial arrhythmias in isolated left ventricular non-compaction patients, while ventricular clotting is facilitated by myocardial trabeculation (7)(8)(9).…”

Section: Discussion and Bibliographic Reviewmentioning

confidence: 99%

Left Ventricular Non-compaction Cardiomyopathy - A Case Report

Timea

Varga

Elena

et al. 2015

Acta Medica Marisiensis

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Background: Left non-compaction cardiomyopathy (LVNC) or "spongy myocardium" is a relatively rare primary genetic cardiomyopathy, characterized by prominent wall trabeculations and intertrabecular recesses which communicate with the ventricular cavity. It appears in isolated form or coexists with other congenital heart diseases and/or systemic abnormalities. Material and method: A 28-year-old woman was admitted with exertional dyspnoea, palpitations, non-specific chest pain and progressive fatigue on exertion. In her family history sudden cardiac-related deaths at young age are present. Cardiovascular system examination revealed tachycardia, intermittent extrabeats.The rest EKG showed sinusal tachycardia (105 bpm), negative T-waves in DII, DIII, aVF, V4-V6. Consecutive 24 hours Holter EKG monitoring revealed nonsustained ventricular tachycardia, paroxysmal atrial fibrillation, isolated ventricular extrasystoles. Echocardiography showed left ventricular systolic dysfunction (LVEF:30-35%), slight LV enlargement, normal right ventricle and small left ventricle (LV) trabeculae in the apical area. Cardiac MRI demonstrated dilated LV and the presence of the trabeculations of LV walls suggestive for non-compaction cardiomyopathy. A combined treatment for heart failure and cardiac arrhythmias was initiated with good clinical results. Patient was scheduled for an implantable cardioverter defibrillator "life-saving". Conclusions: The symptoms of heart failure and cardiac arrhythmias should be considered important in apparently healthy young patients. Besides intensive medical treatment is indicated the implantation of an ICD "life-saving" and in advanced cases heart transplantation. Even if the electrocardiographic findings are non specific for noncompaction, a complete diagnostic evaluation is important, including sophisticated imaging techniques, a screening of first-degree relatives, and an extensive clinical, and genetic appreciation by a multidisciplinary team.

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Left Ventricular Noncompaction

Cited by 96 publications

References 104 publications

Fractal Analysis of Myocardial Trabeculations in 2547 Study Participants: Multi-Ethnic Study of Atherosclerosis

Fractal Analysis of Myocardial Trabeculations in 2547 Study Participants: Multi-Ethnic Study of Atherosclerosis

Endocardial Notch Signaling in Cardiac Development and Disease

Left Ventricular Non-compaction Cardiomyopathy - A Case Report

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