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Abstract：
Background: In arrhythmogenic right ventricular cardiomyopathy (ARVC) non-invasive scar evaluation is not included among the diagnostic criteria or the predictors of ventricular arrhythmias (VA) and sudden death (SD). Computed tomography (CT) has excellent spatial resolution and allows a clear distinction between myocardium and fat; thus, it has great potential for the evaluation of myocardial scar in ARVC. Objective: The objective of this study is to evaluate the feasibility, and the diagnostic and prognostic value of semi-automated quantification of right ventricular (RV) fat replacement from CT images. Methods: An observational case-control study was carried out including 23 patients with a definite (19) or borderline (4) ARVC diagnosis and 23 age- and sex-matched controls without structural heart disease. All patients underwent contrast-enhanced cardiac CT. RV images were semi-automatically reconstructed with the ADAS-3D software (ADAS3D Medical, Barcelona, Spain). A fibrofatty scar was defined as values of Hounsfield Units (HU) <-10. Within the scar, a border zone (between -10 HU and -50 HU) and dense scar (<-50 HU) were distinguished. Results: All ARVC patients had an RV scar and all scar-related measurements were significantly higher in ARVC cases than in controls (p < 0.001). The total scar area and dense scar area showed no overlapping values between cases and controls, achieving perfect diagnostic performance (sensitivity and specificity of 100%). Among ARVC patients, 16 (70%) had experienced sustained VA or aborted SD. Among all clinical, ECG and imaging parameters, the dense scar area was the only one with a statistically significant association with VA and SD (p = 0.003). Conclusions: In ARVC, RV myocardial fat quantification from CT is feasible and may have considerable diagnostic and prognostic value.
摘要：
背景：在致心律失常性右心室心肌病（ARVC）中，无创性瘢痕评估不包括在室性心律失常（VA）和猝死（SD）的诊断标准或预测因子中。计算机断层扫描（CT）具有出色的空间分辨率，可以明确区分心肌和脂肪；因此，它对ARVC中心肌瘢痕的评估具有很大的潜力。目的：本研究的目的是评估可行性，以及从CT图像中半自动定量右心室（RV）脂肪置换的诊断和预后价值。方法：进行了一项观察性病例对照研究，包括23例明确（19例）或临界（4例）ARVC诊断的患者和23例年龄和性别匹配的无结构性心脏病对照。所有患者均行对比增强心脏CT检查。用ADAS-3D软件(ADAS3DMedical，巴塞罗那,西班牙)。纤维脂肪瘢痕定义为Hounsfield单位(HU)的值<-10。在伤疤里，区分为边界区（-10HU和-50HU之间）和致密疤痕（<-50HU）。结果：所有ARVC患者均有RV瘢痕，ARVC患者的所有瘢痕相关测量值均明显高于对照组（p<0.001）。总瘢痕面积和致密瘢痕面积显示病例与对照无重叠值。实现完美的诊断性能（灵敏度和特异性为100％）。在ARVC患者中，16人（70％）经历了持续的VA或中止的SD。在所有临床中，心电图和成像参数，致密瘢痕区域是唯一与VA和SD有统计学显著关联的区域(p=0.003).结论：在ARVC中，CT定量RV心肌脂肪是可行的，可能具有相当大的诊断和预后价值。
