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      A systematic review and meta-analysis of the prevalence of left ventricular non-compaction in adults

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          Abstract

          To assess the reported prevalence of left ventricular non-compaction (LVNC) in different adult cohorts, taking in to consideration the role of diagnostic criteria and imaging modalities used.

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          Most cited references59

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          Isolated noncompaction of left ventricular myocardium. A study of eight cases.

          Isolated noncompaction of left ventricular myocardium is a rare disorder of endomyocardial morphogenesis characterized by numerous, excessively prominent ventricular trabeculations and deep intertrabecular recesses. This study comprised eight cases, including three at necropsy. Ages ranged from 11 months to 22.5 years, with follow-up as long as 5 years. Gross morphological severity ranged from moderately abnormal ventricular trabeculations to profoundly abnormal, loosely compacted trabeculations. Echocardiographic images were diagnostic and corresponded to the morphological appearances at necropsy. The depths of the intertrabecular recesses were assessed by a quantitative echocardiographic X-to-Y ratio and were significantly greater than in normal control subjects (p less than 0.001). Clinical manifestations of the disorder included depressed left ventricular systolic function in five patients, ventricular arrhythmias in five, systemic embolization in three, distinctive facial dysmorphism in three, and familial recurrence in four patients. We conclude that isolated noncompaction of left ventricular myocardium is a rare if not unique disorder with characteristic morphological features that can be identified by two-dimensional echocardiography. The incidence of cardiovascular complications is high. The disorder may be associated with facial dysmorphism and familial recurrence.
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            Measurement of trabeculated left ventricular mass using cardiac magnetic resonance imaging in the diagnosis of left ventricular non-compaction.

            To describe a method for measuring trabeculated left ventricular (LV) mass using cardiac magnetic resonance imaging and to assess its value in the diagnosis of left ventricular non-compaction (LVNC). Between January 2003 and 2008, we prospectively included 16 patients with LVNC. During the mean period, we included 16 patients with dilated cardiomyopathy (DCM), 16 patients with hypertrophic cardiomyopathy (HCM), and 16 control subjects. Left ventricular volumes, LV ejection fraction, and trabeculated LV mass were measured in the four different populations. The percentage of trabeculated LV mass was almost three times higher in the patients with LVNC (32 +/- 10%), compared with those with DCM (11 +/- 4%, P < 0.0001), HCM (12 +/- 4%, P < 0.0001), and controls (12 +/- 5%, P < 0.0001). A value of trabeculated LV mass above 20% of the global mass of the LV predicted the diagnosis of LVNC with a sensitivity of 93.7% [95% confidence interval (CI), 71.6-98.8%] and a specificity of 93.7% (95% CI, 83.1-97.8%; kappa = 0.84). The method described is reproducible and provides an assessment of the global amount of LV trabeculation. A trabeculated LV mass above 20% of the global LV mass is highly sensitive and specific for the diagnosis of LVNC.
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              Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders.

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                Author and article information

                Journal
                European Heart Journal
                Oxford University Press (OUP)
                0195-668X
                1522-9645
                May 29 2019
                May 29 2019
                Affiliations
                [1 ]Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, The University of Sydney, Sydney 2050, New South Wales, Australia
                [2 ]Sydney Medical School, Faculty of Medicine and Health, The University of Sydney, Sydney 2050, New South Wales, Australia
                [3 ]Department of Cardiology, Royal Prince Alfred Hospital, Sydney 2050, New South Wales, Australia
                [4 ]Sydney School of Public Health, Faculty of Medicine and Health, The University of Sydney, Sydney 2050, New South Wales, Australia
                [5 ]Wiser Healthcare, Sydney 2050, New South Wales, Australia
                Article
                10.1093/eurheartj/ehz317
                31143950
                65c257a8-4991-473a-987a-84dfa3ba316e
                © 2019

                https://academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model

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