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目的回顾性分析27例致心律不齐性右室型心肌病(ARVC)的磁共振成像(MRI)表现,探讨MRI在ARVC的诊断与预后判断中的价值。方法按照1994年WHO关于ARVC的诊断标准,2004年10月至2006年6月共27例临床诊断或病理确诊为ARVC(6例行心脏移植术),男21例,女6例,平均年龄37.4(15～67)岁。采用1.5T超导MRI扫描仪对心脏形态(脂肪浸润、房室大小)、功能(室壁局部与整体运动功能)、心肌灌注与心肌存活等方面进行综合评价。结果形态学:88.89%(24/27)的病例MRI提示心肌脂肪浸润,62.96%(17/27)右室壁变薄,62.96%(17/27)右室心尖肌小梁明显粗乱,66.67%(18/27)右室流出道扩张,51.85%(14/27)右室心尖扩张,66.67%(18/27)右室下壁及游离壁扩张,40.74%(11/27)合并右房增大。心脏功能:18.52%(5/27)的病例右室局部运动功能异常,70.37%(19/27)整体运动功能异常,右室平均射血分数(EF)35%。40.74%(11/27)的患者合并左室扩大并室壁收缩运动明显减弱。心肌首过灌注示10.52%(2/19)的患者左室受累,36.84%(7/19)的患者左室和右室壁出现异常强化,提示心肌纤维或胶原变性。右室壁强化区域主要位于右室游离壁和右室流出道肌壁,左室则主要位于左室侧壁,少数合并左室心尖或室间隔,5例左室侧壁异常强化经术后病理证实为纤维组织。仅1例表现为右室流出道增宽,但左室心肌显著变薄,收缩运动明显减弱;有3例右室MRI无阳性表现,其中2例左室侧壁室壁变薄并运动异常,延迟显像为异常强化,另1例表现为类似扩张型心肌病样改变。结论MRI高度的软组织对比与多序列成像可对ARVC进行全面诊断与预后评价,但少数以左室异常表现为主而无明显或仅轻微右室异常的病例,MRI易误诊,其左室侧壁段的纤维化为ARVC相对特征表现。右室整体运动异常、广泛纤维脂肪浸润、合并左室扩张并运动异常为其预后不良的指标。
Objective To retrospectively analyze the magnetic resonance imaging (MRI) findings of 27 cases of arrhythmogenic right ventricular cardiomyopathy (ARVC) and investigate the value of MRI in the diagnosis and prognosis of ARVC. Methods According to the WHO diagnostic criteria of ARVC in 1994, 27 cases of ARVC (6 cases underwent cardiac transplantation) were diagnosed or pathologically diagnosed in 27 cases from October 2004 to June 2006. There were 21 males and 6 females, with an average age of 37.4 (15 ~ 67) years old. A 1.5T superconducting MRI scanner was used to evaluate the cardiac morphology (fat infiltration, atrioventricular size), function (regional and global motor function), myocardial perfusion and myocardial viability. Results Morphology: 88.89% (24/27) cases of myocardial infarction showed myocardial infiltration, 62.96% (17/27) of right ventricular wall thinning, 62.96% (17/27) right ventricular apex trabecular obstruction, 66.67 The right ventricular outflow tract was dilated in% (18/27), the apical dilatation of right ventricle in 51.85% (14/27), the dilatation of the inferior wall and the free wall in 66.67% (18/27) and the extension of the right ventricle in 40.74% (11/27) Increase. Cardiac function: Right ventricular regional motor function was abnormal in 18.52% (5/27) cases, global motor function was abnormal in 70.37% (19/27), and right ventricular ejection fraction (EF) was 35%. 40.74% (11/27) of patients with left ventricular enlargement and ventricular contraction was significantly reduced. Left ventricular involvement was observed in 10.52% (2/19) of patients with first myocardial perfusion, and abnormal enhancement of left ventricle and right ventricular wall was observed in 36.84% (7/19) of patients, suggesting degeneration of myocardial fibers or collagen. Right ventricular wall enhancement area mainly located in the right ventricular free wall and right ventricular outflow tract muscle wall, left ventricular mainly located in the left ventricular wall, a few with left ventricular apex or ventricular septum, 5 cases of left ventricular wall abnormalities enhanced by postoperative pathology Confirmed as fibrous tissue. Only one case showed right ventricular outflow tract widened, but left ventricular myocardial thinning significantly reduced contractility; 3 cases of right ventricular MRI showed no positive, of which 2 cases of left ventricular wall thinning and abnormal motion, Delayed imaging was abnormal enhancement, the other case showed similar dilated cardiomyopathy-like changes. Conclusion MRI-based soft tissue contrast and multi-sequence imaging can provide a comprehensive diagnosis and prognosis evaluation of ARVC. However, a small number of cases with abnormal left ventricular manifestations without obvious or slight right ventricular abnormalities are easily misdiagnosed by MRI. The left ventricular wall Fibrosis is the relative characteristic of ARVC. Right ventricular total abnormalities, extensive fibrous fat infiltration, combined with left ventricular dilatation and motor abnormalities as an indicator of poor prognosis.