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法洛四联症修复术后幼儿存在左心室旋转异常,且与右心室扩张无关。

Abnormalities in Left Ventricular Rotation Are Inherent in Young Children with Repaired Tetralogy of Fallot and Are Independent of Right Ventricular Dilation.

作者信息

Karnik Ruchika, Uppu Santosh C, Tozzi Meghan, Doucette John, Lytrivi Irene D, Geiger Miwa, Klas Berthold, Parness Ira A, Shenoy Rajesh, Rajagopal Hari, Srivastava Shubhika

机构信息

Division of Pediatric Cardiology, Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, USA.

Department of Environmental Medicine and Public Health, Icahn School of Medicine at Mount Sinai, New York, USA.

出版信息

Pediatr Cardiol. 2018 Aug;39(6):1172-1180. doi: 10.1007/s00246-018-1877-9. Epub 2018 Apr 11.

Abstract

Left ventricular (LV) dysfunction is a risk factor for adverse outcomes in older children and adults with repaired Tetralogy of Fallot (rToF). Pulmonary regurgitation (PR), right ventricular (RV) dilation, and dysfunction have been shown to result in abnormal LV myocardial mechanics and dysfunction. The aim of our study was to evaluate LV rotational mechanics, especially apical rotation in young children with rToF with and without RV dilation. This is a retrospective, single center study in 28 asymptomatic young children with rToF (16 with RV dilation; 12 without RV dilation); 29 age-matched normal controls. RV and LV systolic and diastolic function was studied using conventional two-dimensional echocardiography (2DE) and speckle tracking echocardiography (STE). Rotational mechanics studied included basal and apical rotation (BR, AR), peak twist (calculated by difference between the apical and basal rotation), twist rate (TR), and untwist rate (UnTR). The mean age of the cohort was 4.7 years (± 2.3). Abnormal AR, BR, TR, and UnTR were noted in patients with rToF. The abnormalities were significant in magnitude as well as the direction of rotation; more pronounced in the absence of RV dilation. LV systolic and diastolic dysfunction as evidenced by abnormal AR and degree of untwist is inherent in rToF and not associated with RV dilation in rToF children. Abnormal BR may reflect a lack of maturation to adult type of rotational mechanics. Further longitudinal studies are required to study the progression of these abnormalities and their correlation with clinical outcomes.

摘要

左心室(LV)功能障碍是大龄儿童和法洛四联症修复术后(rToF)成人不良预后的一个危险因素。肺反流(PR)、右心室(RV)扩张及功能障碍已被证实会导致左心室心肌力学异常和功能障碍。我们研究的目的是评估rToF患儿无论有无右心室扩张时的左心室旋转力学,尤其是心尖旋转。这是一项针对28例无症状rToF患儿(16例有右心室扩张;12例无右心室扩张)的回顾性单中心研究;29例年龄匹配的正常对照。使用传统二维超声心动图(2DE)和斑点追踪超声心动图(STE)研究右心室和左心室的收缩及舒张功能。研究的旋转力学指标包括心底和心尖旋转(BR、AR)、峰值扭转(通过心尖和心底旋转的差值计算得出)、扭转率(TR)和解旋率(UnTR)。该队列的平均年龄为4.7岁(±2.3)。rToF患者存在异常的AR、BR、TR和UnTR。这些异常在幅度和旋转方向上均很显著;在无右心室扩张时更为明显。rToF患儿中,AR异常和解旋程度所证实的左心室收缩和舒张功能障碍是rToF所固有的,与右心室扩张无关。异常的BR可能反映了向成人型旋转力学的成熟不足。需要进一步的纵向研究来探讨这些异常的进展及其与临床结局的相关性。

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