Wu Qingyu, Pan Guangyu, Li Hongyin, Kong Xiangchen
Heart Center, First Hospital of Tsinghua University, Beijing, China.
Heart Center, First Hospital of Tsinghua University, Beijing, China.
J Thorac Cardiovasc Surg. 2014 Oct;148(4):1454-1458.e1. doi: 10.1016/j.jtcvs.2014.02.075. Epub 2014 Mar 2.
Ebstein's anomaly with isolated anterior leaflet downward displacement is rare and has not been reported in the literature to our knowledge. In this article, our experience of the surgical treatment in 6 cases with this anomaly is reported.
From November 2005 to November 2013, 6 patients (3 male, 3 female, aged 2-39 years) with Ebstein's anomaly and isolated anterior leaflet downward displacement received anatomic repair at the First Hospital of Tsinghua University. The diagnosis was made by echocardiography and confirmed at operation. Surgery was performed under hypothermic cardiopulmonary bypass. Surgical technique included excision of a huge atrialized portion of the right ventricle located in the anterior wall of the heart; reconstruction of the right ventricle by repairing the "V"-shaped defect left by the excision procedure; detachment, repair, and reimplantation of the anterior leaflet; and reconstitution of the right atrioventricular connection. Intraoperative transesophageal echocardiography was used to evaluate the position, morphology, structure, and function of the tricuspid valve, as well as right ventricular function.
Five patients were discharged uneventfully, and 1 patient died of postoperative pneumonia. At follow-up from 2 months to 7 years, no notable tricuspid valve regurgitation or stenosis was found and all patients were maintaining a normal lifestyle. The 27-year-old female patient gave birth to a normal infant uneventfully 3 years after surgery.
Ebstein's anomaly with isolated anterior leaflet downward displacement is a complex and severe abnormality, and has several unique anatomic and clinical features and specific surgical requirements. Preoperative diagnosis can be made by clinical investigation and echocardiography. Excellent results can be achieved by anatomic correction.
据我们所知，孤立性前叶下移的埃布斯坦畸形罕见，文献中尚无报道。本文报告我们对6例该畸形患者的手术治疗经验。
2005年11月至2013年11月，6例（3男3女，年龄2至39岁）孤立性前叶下移的埃布斯坦畸形患者在清华大学第一医院接受了解剖修复手术。诊断通过超声心动图做出，并在手术中得到证实。手术在低温体外循环下进行。手术技术包括切除位于心脏前壁的巨大右心室房化部分；通过修复切除术后留下的“V”形缺损来重建右心室；分离、修复和重新植入前叶；以及重建右房室连接。术中使用经食管超声心动图评估三尖瓣的位置、形态、结构和功能，以及右心室功能。
5例患者顺利出院，1例患者死于术后肺炎。随访2个月至7年，未发现明显的三尖瓣反流或狭窄，所有患者生活方式正常。1例27岁女性患者术后3年顺利产下一名正常婴儿。
孤立性前叶下移的埃布斯坦畸形是一种复杂且严重的异常，具有若干独特的解剖和临床特征以及特定的手术要求。术前诊断可通过临床检查和超声心动图做出。通过解剖矫正可取得良好效果。
