Fogel Mark A, Weinberg Paul M, Harris Matthew, Rhodes Larry
Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia, The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA.
Am J Cardiol. 2006 Apr 15;97(8):1232-7. doi: 10.1016/j.amjcard.2005.11.045. Epub 2006 Mar 6.
Cardiac magnetic resonance (CMR) has been helpful in adults in the diagnosis of arrhythmogenic right ventricular dysplasia. Short of direct surgical observation or autopsy, no gold standard exists. CMR diagnostic criteria include right atrial and ventricular dilation, regional right ventricular (RV) wall motion abnormalities, outflow tract ectasia, and myocardial fatty infiltration. To determine whether adult diagnostic criteria are useful in children referred for CMR for this diagnosis, the images and records of 81 patients (aged 11.5 +/- 5.5 years) over an 8-year period were reviewed. Histories included ventricular tachycardia, palpitations, dilated right ventricle, syncope, near sudden death, or family history of RV dysplasia. Four families were studied with parents who had RV dysplasia diagnosed by surgery, explanted heart, or CMR. CMR imaging included T1-weighted imaging, cine, 1-dimensional RV myocardial tagging, and phase-encoded velocity mapping, and 2 patients underwent delayed-enhancement CMR. Only 1 of the 81 patients met 5 of the criteria. None of the others met >2 of the criteria, and only 2 patients met 1 or 2 criteria. For questionable regional wall motion abnormalities, RV myocardial tagging was helpful. In conclusion, CMR of patients with a history suspicious for the diagnosis of RV dysplasia is a low-yield test in children. This may be due to the evolving nature of the disease, which does not manifest itself from a morphologic or ventricular-function standpoint until later in development. Follow-up studies as patients age may be advantageous.
心脏磁共振成像（CMR）对成人诊断致心律失常性右心室发育不良很有帮助。除了直接手术观察或尸检外，不存在金标准。CMR诊断标准包括右心房和心室扩张、右心室（RV）局部室壁运动异常、流出道扩张和心肌脂肪浸润。为了确定成人诊断标准对因该诊断接受CMR检查的儿童是否有用，我们回顾了8年期间81例患者（年龄11.5±5.5岁）的图像和记录。病史包括室包括室性心动过速、心悸、右心室扩张、晕厥、濒死感或右心室发育不良家族史。对4个家庭进行了研究，其父母经手术、心脏移植或CMR诊断为右心室发育不良。CMR成像包括T1加权成像、电影成像、一维右心室心肌标记和相位编码速度图，2例患者接受了延迟强化CMR检查。81例患者中只有1例符合5条标准。其他患者均未超过2条标准，只有2例患者符合1条或2条标准。对于可疑的局部室壁运动异常，右心室心肌标记很有帮助。总之，有右心室发育不良可疑诊断病史患者的CMR检查在儿童中阳性率较低。这可能是由于该疾病不断演变的性质，从形态学或心室功能角度来看，直到发育后期才会表现出来。随着患者年龄增长进行随访研究可能会有帮助。
