BACKGROUND 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. METHODS 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. RESULTS 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). CONCLUSIONS 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.

中文翻译：

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青少年心律失常性右心室心肌病的超声心动图参数与右心室重构和心肌功能与改良的特别工作组标准的关系。

背景技术超声心动图指标（包括已由改良的工作组标准（mTFC）使用的那些指标，以及诸如应变成像等指标）在青春期识别心律失常性右室心肌病（ARVC）的用途尚不十分清楚。方法回顾性分析120例ARVC（13±4岁）青少年的超声心动图。根据mTFC，将患者分为明确的（n = 38），临界（n = 39）或可能的（n = 43）ARVC。将结果与35名健康对照组进行比较。分析了mTFC超声心动图参数，以及综合的右心室（RV）和左心室功能评估，包括mTFC中未包括的参数，例如脉冲波组织多普勒和RV二维散斑应变。结果与可能的，边缘的或确定的ARVC的患者相比，针对体表面积的mTFC参数异常明显高于右胸骨旁长轴视野的右室流出道对照。与对照组相比，患者的RV舒张末期直径明显更大，这一差异随着ARVC的可能性而增加。左心室射血分数，三尖瓣环峰值收缩期偏移，收缩期和舒张期脉冲波组织多普勒成像指数类似于所有组的控件。在具有明确ARVC（-21±4％）和疾病亚组的患者中，平均和节段性RV峰值纵向收缩应变显着低于对照组（-25±3％）。多变量风险分析显示，RV应变降低与ARVC诊断及其可能性显着相关（多变量比值比[95％CI] = 1.23 [1.1-1.37]； P <0.001），与舒张末期末梢直径增加的三分之一有关。 RV（多因素优势比[95％CI] = 1.51 [1.33-1.72]； P <0.001）。结论在患者和对照组之间以及不同的诊断组之间，mTFC超声心动图标准存在显着差异。但是，在我们的队列中，大多数青春期ARVC患者无法满足当前的超声心动图mTFC，尤其是当按体表面积指数进行时。RV顶端尺寸和应变的测量可能会增加超声心动图对ARVC的诊断率。001），而舒张末期舒张末期直径在RV的顶端增加（多变量优势比[95％CI] = 1.51 [1.33-1.72]； P <0.001）。结论在患者和对照组之间以及不同的诊断组之间，mTFC超声心动图标准存在显着差异。但是，在我们的队列中，大多数青春期ARVC患者无法满足当前的超声心动图mTFC，尤其是当按体表面积指数进行时。RV顶端尺寸和应变的测量可能会增加超声心动图对ARVC的诊断率。001），而舒张末期舒张末期直径在RV的顶端增加（多变量优势比[95％CI] = 1.51 [1.33-1.72]； P <0.001）。结论在患者和对照组之间以及不同的诊断组之间，mTFC超声心动图标准存在显着差异。但是，在我们的队列中，大多数青春期ARVC患者无法满足当前的超声心动图mTFC，尤其是当按体表面积指数进行时。RV顶端尺寸和应变的测量可能会增加超声心动图对ARVC的诊断率。大多数青少年ARVC患者无法满足当前的超声心动图mTFC要求，尤其是在按体表面积进行索引时。RV顶端尺寸和应变的测量可能会增加超声心动图对ARVC的诊断率。大多数青少年ARVC患者无法满足当前的超声心动图mTFC要求，尤其是在按体表面积进行索引时。RV顶端尺寸和应变的测量可能会增加超声心动图对ARVC的诊断率。