Department of Pediatrics, Labatt Family Heart Centre (G.E.P., L.G.-W., M.H., M.F., P.C., C.S., W.H., C.-P.S.F., C.M., L.M., R.H., M.K.F.), Hospital for Sick Children, University of Toronto, ON, Canada.
NIHR Cardiovascular Biomedical Research Centre, Bristol Heart Institute, United Kingdom (G.E.P.).
Circ Cardiovasc Imaging. 2019 Apr;12(4):e007693. doi: 10.1161/CIRCIMAGING.118.007693.
The usefulness of echocardiographic indices, including those already used by modified Task Force Criteria (mTFC), and others such as strain imaging, to identify arrhythmogenic right ventricular cardiomyopathy (ARVC) in adolescence is not well established.
Echocardiograms from 120 adolescents investigated for ARVC (13±4 years) were retrospectively analyzed. According to the mTFC, patients were classified into definite (n=38), borderline (n=39), or possible (n=43) ARVC. Results were compared with 35 healthy controls. mTFC echocardiographic parameters were analyzed, as well as comprehensive right ventricular (RV) and left ventricular assessment of function including parameters not included in mTFC such as pulsed-wave tissue Doppler and RV 2-dimensional speckle strain.
mTFC parameters indexed for body surface area were significantly more abnormal in patients with possible, borderline, or definite ARVC compared with controls for parasternal long-axis view of the RV outflow tract. RV end-diastolic diameters were significantly larger in patients versus controls, a difference that increased with likelihood of ARVC. Left ventricular ejection fraction, tricuspid annular peak systolic excursion, and systolic and diastolic pulsed-wave tissue Doppler imaging indices were similar to controls for all groups. Average and segmental RV peak longitudinal systolic strain was significantly lower in patients with definite ARVC (-21±4%) and disease subgroups versus controls (-25±3%). Multivariable risk analysis showed that reduced RV strain was significantly associated with ARVC diagnosis and its likelihood (multivariable odds ratio [95% CI]=1.23 [1.1-1.37]; P<0.001) as was increased end-diastolic diameter at the apical third of the RV (multivariable odds ratio [95% CI]=1.51 [1.33-1.72]; P<0.001).
mTFC echocardiographic criteria are significantly different between patients and controls and between the different diagnostic groups. However, in our cohort, current echocardiographic mTFC are not met by the majority of adolescent ARVC patients, particularly when indexed to body surface area. Measurement of RV apical dimensions and strain may increase the diagnostic yield of echocardiography for ARVC.
超声心动图指标的有用性，包括改良工作组标准（mTFC）中已经使用的指标以及应变成像等其他指标，用于识别青少年心律失常性右心室心肌病（ARVC）的作用尚未得到充分证实。
回顾性分析了 120 名接受 ARVC 检查的青少年（13±4 岁）的超声心动图。根据 mTFC，患者被分为明确（n=38）、边界（n=39）或可能（n=43）ARVC。将结果与 35 名健康对照进行比较。分析了 mTFC 超声心动图参数，以及全面的右心室（RV）和左心室功能评估，包括 mTFC 未包含的参数，如脉冲波组织多普勒和 RV 二维斑点应变。
与对照组相比，可能、边界或明确 ARVC 患者的 RV 流出道胸骨旁长轴观的 mTFC 参数指标与对照组相比明显更异常。RV 舒张末期直径在患者中明显大于对照组，这种差异随着 ARVC 的可能性而增加。左心室射血分数、三尖瓣环收缩期峰值位移以及收缩期和舒张期脉冲波组织多普勒成像指数在所有组中均与对照组相似。明确 ARVC 患者（-21±4%）和疾病亚组患者的平均和节段 RV 纵向收缩峰值应变明显低于对照组（-25±3%）。多变量风险分析显示，RV 应变降低与 ARVC 诊断及其可能性显著相关（多变量优势比[95%CI]=1.23[1.1-1.37]；P<0.001），RV 舒张末期直径增加也与 ARVC 诊断显著相关（多变量优势比[95%CI]=1.51[1.33-1.72]；P<0.001）。
mTFC 超声心动图标准在患者与对照组之间以及不同诊断组之间存在显著差异。然而，在我们的队列中，大多数青少年 ARVC 患者的超声心动图 mTFC 目前都不符合标准，特别是当根据体表面积进行索引时。测量 RV 心尖部尺寸和应变可能会提高超声心动图对 ARVC 的诊断效果。
