A Preliminary Study of Left Ventricular Rotational Mechanics in Children with Noncompaction Cardiomyopathy: Do They Influence Ventricular Function?

被引:9
作者
Nawaytou, Hythem M. [1 ,2 ]
Montero, Andrea E. [1 ]
Yubbu, Putri [1 ]
Calderon-Anyosa, Renzo J. C. [1 ]
Sato, Tomoyuki [1 ]
O'Connor, Matthew J. [1 ]
Miller, Kelley D. [1 ]
Ursell, Philip C. [3 ]
Hoffman, Julien I. E. [2 ]
Banerjee, Anirban [1 ]
机构
[1] Childrens Hosp Philadelphia, Div Cardiol, 8NW-53,3401 Civ Ctr Blvd, Philadelphia, PA 19104 USA
[2] Univ Calif San Francisco, Div Pediat Cardiol, San Francisco, CA 94143 USA
[3] Univ Calif San Francisco, Dept Pathol, San Francisco, CA 94140 USA
关键词
Cardiomyopathy; Mechanics; Noncompaction; Pediatrics; Torsion; NON-COMPACTION; DILATED CARDIOMYOPATHY; MAGYAR-PATH; ECHOCARDIOGRAPHY; CLASSIFICATION; EPIDEMIOLOGY; MYOCARDIUM; DIAGNOSIS; INSIGHTS; OUTCOMES;
D O I
10.1016/j.echo.2018.02.015
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Background: Current diagnostic criteria for noncompaction cardiomyopathy (NCC) lack specificity, and the disease lacks prognostic indicators. Reverse apical rotation (RAR) with abnormal rotation of the cardiac apex in the same clockwise direction as the base has been described in adults with NCC. The aim of this study was to test the hypothesis that RAR might differentiate between symptomatic NCC and benign hypertrabeculations and might be associated with ventricular dysfunction. Methods: Echocardiograms from 28 children with NCC without cardiac malformations were prospectively compared with those from 29 age-matched normal control subjects. A chart review was performed to identify the patients' histories and clinical characteristics. Speckle-tracking was used to measure longitudinal strain, circumferential strain, and rotation. Results: RAR occurred in 39% of patients with NCC. History of left ventricular (LV) dysfunction or arrhythmia was universal in, but not exclusive to, patients with RAR. Patients with RAR had lower LV longitudinal strain but similar ejection fractions compared with patients without RAR (median, -15.6% [interquartile range, -12.9% to -19.3%] vs -19% [interquartile range, -14.5% to -21.9%], P < .01; 53% [interquartile range, 43% to 68%] vs 61% [interquartile range, 58% to 67%], P = .08). Only a pattern of contraction with RAR, early arrest of twisting by mid-systole, and premature untwisting was associated with lower ejection fraction (46%; interquartile range, 43% to 52%; P = .006). Conclusions: RAR is not a sensitive but is a specific indicator of complications in children with NCC. Therefore, RAR may have prognostic rather than diagnostic value. Premature untwisting of the left ventricle during ejection may be an even more worrisome indicator of LV dysfunction.
引用
收藏
页码:951 / 961
页数:11
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