Abstract

Introduction: Left ventricular noncompaction (LVNC) is a rare form of cardiomyopathy in children, characterized by abnormal endocardial myofiber arrangement. This may result in an altered pattern of LV rotation depicted by a “roller pump” like motion and described as rigid body rotation (RBR). This pattern, is characterized by the apex and the base rotating in the same direction. Hypothesis: To describe the LV rotational patterns in children with LVNC using speckle tracking echocardiography and detect the relationship between the different rotational patterns and measures of LV systolic function. Methods: We prospectively studied 19 children (age 0.9 to 17.8 years) with LVNC and compared them with age-matched controls. Apical and basal rotations, peak twist and peak torsion were analyzed as indices of systolic function. Peak apical recoil rate was measured as an index of diastolic function. Results: Children with LVNC had significantly lower apical rotation (6.26°± 3.09°, 8.59°± 2.19° p=0.01) than controls. In 7 out of 19 (36.8%) patients a pattern of RBR was detected. Twelve patients exhibited the typical “wringing” motion of the LV, where the apex rotated counterclockwise and the base clockwise. Patients with LVNC and RBR had significantly lower ejection fraction than patients with LVNC without RBR (49.1 ± 9.2% vs 60.3 ± 10.2%, p<0.05). There were no significant differences in the remainder of the systolic and diastolic indices between LVNC patients and controls. Conclusions: Children with LVNC can exhibit an abnormal LV rotational pattern characterized by RBR that is associated with impaired systolic function.

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