Background: The incidence of Ebstein's anomaly is extremely low, and except for the Mayo Clinic, no cardiac center has reported on a sufficient number of patients. The aim of our study was to report the outcomes of Ebstein's anomaly patients treated with tricuspid valvuloplasty (TVP) or tricuspid valve replacement (TVR).
Methods: TVP or TVR was performed in 245 patients from July 2006 to April 2016. We reviewed patients' records and contacted patients via outpatient service and over the telephone.
Results: The mean follow-up time was 43.6 ± 32.6 months, and 224 (91.4%) patients underwent follow-up. The mean operative age was 31.2 ± 15.7 years. TVR was performed in 23 patients, and TVP was performed in 201 patients. The 30-day mortality rate was 1.3%, and the overall survival rate was 97.9% at 5 and 10 years. The early mortality rate of the TVP group was lower than that of the TVR group (0.5% vs. 8.7%, P = 0.028), and the overall mortality rate of the TVP group was lower than that of the TVR group, without statistical significance (1.0% vs. 8.7%). After propensity score matching, the rates of mortality and New York Heart Association class ≥ III were lower in the TVP group than those in the TVR group without statistical significance. Seven patients with Type B Wolff-Parkinson-White (WPW) syndrome underwent one-stage surgery, and arrhythmias disappeared. Six patients suffered from episodes of left ventricular outflow tract obstruction (LVOTO) during surgery. Severe LVOTO could be treated with reoperation of the atrialized right ventricle.
Conclusions: Ebstein's anomaly patients treated with TVP or TVR can experience optimal outcomes with midterm follow-up. However, TVP should be the first-choice treatment. Optimal outcomes can be obtained from one-stage operation in patients with Type B WPW syndrome. Severe LVOTO during surgery might be related to improper operation of the atrialized right ventricle.
三尖瓣下移畸形患者行三尖瓣成形术或置换术后单中心中期疗效观察摘要背景：三尖瓣下移畸形的发生率极低。除梅奥诊所外，其他心脏中心报道的三尖瓣下移畸形患者数量仍较少。本研究的主要目的是报道三尖瓣下移畸形患者行三尖瓣成形术或三尖瓣置换后的近中期结果。 方法：从2006年7月到2016年4月，共有245例三尖瓣下移畸形患者行三尖瓣成形术或三尖瓣置换术。我们通过复习患者的病历资料，门诊随访和电话调查的方式进行随访。 结果：平均随访时间43.6±32.6月。共随访到224例患者，随访率为91.4%。平均手术年龄31.2±15.7岁。其中行三尖瓣置换术患者23例，行三尖瓣成形术患者201例。30天内死亡率为1.3%，5年及10年总生存率为97.9%。三尖瓣成形术患者早期死亡率低于三尖瓣置换术组（0.5% vs. 8.7%, Fisher's exact test, P = 0.028）。三尖瓣成形术组总死亡率低于三尖瓣置换术组（1.0% vs. 8.7%），但并无统计学差异。经倾向性评分匹配后，三尖瓣成形组的死亡率、纽约心功能分级≥III级发生率低于三尖瓣置换组，但无统计学差异。7例患者行同期处理三尖瓣下移畸形和B型预激综合征，术后心律失常消失。6例患者于术中出现左室流出道梗阻现象，重度的左室流出道梗阻可以通过再次处理房化右室纠正。 结论：三尖瓣下移畸形患者行三尖瓣成形术或三尖瓣置换术后早中期效果良好，但三尖瓣成形术应为首选；三尖瓣下移畸形合并B型预激的患者可行同期手术，效果良好；三尖瓣下移矫治术中可能出现罕见的左室流出道梗阻，重度的左室流出道梗阻可能与房化右室处理不当相关。.
Keywords: Congenital Heart Disease; Right Heart Failure; Tricuspid Regurgitation; Tricuspid Valve Dysplasia.