Cardiomyopathy
New Classification Scheme of Left Ventricular Noncompaction and Correlation With Ventricular Performance

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Isolated left ventricular noncompaction (LVNC) is an increasingly-recognized cardiomyopathy, and the possibility that it exists as a spectrum of disease has yet to be explored. We sought to determine the prevalence, spectrum, and functional consequences of LVNC; 2 blinded reviewers assessed 500 transthoracic echocardiograms for LVNC for adequate study quality, absence of co-existing cardiomyopathy, and LVNC. If present, the ratio of the maximum linear length of noncompacted to compacted myocardium (NC/C) and the planimetered area of LVNC on apical 4-chamber view were measured. Patients were classified by degree of noncompaction measured by either the NC/C ratio or LVNC area as controls, mild, moderate, and severe; 380 patients were included in the analysis and 60 (15.8%) had evidence of noncompaction. Patients with increasing severity of noncompaction had significantly decreased ejection fractions. In conclusion, these findings indicate that LVNC may be more common than previously recognized and may exist as a spectrum, which can be classified using the NC/C ratio or LVNC area classification schemes.

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Methods

All patients undergoing transthoracic echocardiography at Mount Sinai Medical Center from September 10, 2005 to January 5, 2006 were eligible for inclusion. Of the >4,000 echocardiograms performed during the study period, we randomly selected 500 from daily digitally stored studies. Reviewers selecting patients for study inclusion were blinded to patient demographic and echocardiographic characteristics. After being selected for inclusion, each echocardiogram was reviewed before obtaining

Results

Of the 500 patients originally selected for inclusion in the study, 74 patients (14.8%) were excluded because they had evidence of congenital heart disease, hypertrophic or infiltrative cardiomyopathy, or documented coronary artery disease. An additional 46 patients (9.2%) were excluded because the apex was not adequately visualized for full analysis. Of the remaining 380 patients, 60 (15.8%) had evidence of some degree of noncompaction. There were no significant differences between controls

Discussion

Isolated noncompaction of the left ventricle is a recently described cardiomyopathy, often detected incidentally. We sought to assess the prevalence and spectrum of the disease, correlate the severity of noncompaction with characteristics of left ventricular performance, and evaluate 2 novel classification schemes. LVNC was originally believed to be an extremely rare disorder, with early estimates of prevalence ranging 0.014% to 0.045%,5, 6 although more recent work has estimated the prevalence

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Adam Belanger was supported by a grant from the Doris Duke Clinical Research Foundation, New York, New York.

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