Ammash N M, Warnes C A, Connolly H M, Danielson G K, Seward J B
Division of Cardiovascular Diseases, Mayo Clinic and Foundation, Rochester 55905, USA.
Am Heart J. 1997 Sep;134(3):508-13. doi: 10.1016/s0002-8703(97)70088-7.
The purpose of this study was to determine the most discriminating clinical and echocardiographic features that are most helpful in correctly identifying Ebstein's anomaly of the tricuspid valve from other causes of tricuspid regurgitation. Ebstein's anomaly is an uncommon malformation of the tricuspid valve with diagnostic echocardiographic features. Other cardiac disorders associated with tricuspid valve regurgitation and predominate right-sided heart chamber enlargement can be misdiagnosed as Ebstein's anomaly. All patients who were referred to our institution between 1982 and 1995 with the diagnosis of Ebstein's anomaly but were found to have other abnormalities of the tricuspid value or right ventricle were identified. Their clinical, echocardiographic, and surgical records were reviewed retrospectively. Twenty-two patients (12 males and 10 females), aged 7 to 68 years (mean 33 years), were referred to our institution with the diagnosis of Ebstein's anomaly but were found to have another abnormality that mimicked clinical and diagnostic features of Ebstein's anomaly. The most common initial symptom was exercise intolerance (13 [59%] patients) followed by atrial arrhythmia (seven [32%] patients). Two patients had cyanosis. Three patients had paroxysmal and six had chronic atrial fibrillation/flutter. Cardiomegaly on chest x-ray film was noted in 18 (82%) patients. Referral diagnosis of Ebstein's anomaly had been made by echocardiography (12 patients), cardiac catheterization (four patients), both techniques (five patients), and echocardiography and magnetic resonance imaging (one patient). All 22 patients had predominate right atrial and right ventricular enlargement, and 18 (82%) of 22 patients also had right ventricular dysfunction. However, Ebstein's anomaly was confidently ruled out with repeat comprehensive echocardiography at our institution by establishing (1) absence of significant apical displacement of the septal tricuspid valve leaflet (> or = 8 mm/m2) and (2) lack of a redundant, elongated, anterior tricuspid valve leaflet in all 22 patients (100%). All had significant tricuspid regurgitation caused by tricuspid valve dysplasia (nine patients), tricuspid valve prolapse (four patients), trauma (four patients), right ventricular dysplasia (three patients), endocarditis (one patient), and annular dilation caused by free pulmonary regurgitation (one patient). In all 15 patients who subsequently underwent surgery (tricuspid valve repair [seven patients] or replacement [eight patients]), the absence of Ebstein's anomaly was confirmed. Echocardiographic absence of the characteristic degree of displacement of the septal leaflet of the tricuspid valve (> or = 8 mm/m2) and the presence of a nonelongated, nonredundant anterior tricuspid valve leaflet consistently excluded the diagnosis of Ebstein's anomaly. Under such circumstances, other anomalies of the tricuspid valve or right ventricle were consistently identified. Recognition of the mimics of Ebstein's anomaly had important surgical implications.
本研究的目的是确定最具鉴别力的临床和超声心动图特征，这些特征有助于从其他三尖瓣反流病因中正确识别三尖瓣埃布斯坦畸形。埃布斯坦畸形是一种罕见的三尖瓣畸形，具有诊断性超声心动图特征。与三尖瓣反流相关且以右心腔扩大为主的其他心脏疾病可能被误诊为埃布斯坦畸形。确定了1982年至1995年间转诊至本机构、诊断为埃布斯坦畸形但发现有三尖瓣或右心室其他异常的所有患者。对他们的临床、超声心动图和手术记录进行了回顾性分析。22例患者（12例男性和10例女性），年龄7至68岁（平均33岁），转诊至本机构时诊断为埃布斯坦畸形，但发现有另一种异常，其临床和诊断特征与埃布斯坦畸形相似。最常见的初始症状是运动不耐受（13例[59%]患者），其次是房性心律失常（7例[32%]患者）。2例患者有发绀。3例患者有阵发性房颤，6例患者有慢性房颤/房扑。18例（82%）患者胸部X线片显示心脏扩大。埃布斯坦畸形的转诊诊断通过超声心动图（12例患者）、心导管检查（4例患者）、两种技术（5例患者）以及超声心动图和磁共振成像（1例患者）做出。所有22例患者均以右心房和右心室扩大为主，22例患者中有18例（82%）也有右心室功能障碍。然而，通过在本机构重复进行全面超声心动图检查，确定（1）三尖瓣隔叶无明显心尖移位（>或=8 mm/m2）以及（2）所有22例患者（100%）均无多余、冗长的三尖瓣前叶，从而可靠地排除了埃布斯坦畸形的诊断。所有患者均有由三尖瓣发育异常（9例患者）、三尖瓣脱垂（4例患者）、创伤（4例患者）、右心室发育异常（3例患者）、心内膜炎（1例患者）以及游离肺动脉反流导致的瓣环扩张（1例患者）引起的严重三尖瓣反流。在随后接受手术的所有15例患者（三尖瓣修复[7例患者]或置换[8例患者]）中，均证实不存在埃布斯坦畸形。超声心动图显示三尖瓣隔叶无特征性移位程度（>或=8 mm/m2）以及存在无冗长、无多余的三尖瓣前叶始终排除了埃布斯坦畸形的诊断。在这种情况下，始终能识别出三尖瓣或右心室的其他异常。识别埃布斯坦畸形的模仿者具有重要的手术意义。
