Lim Jae Hong, Lee Chang-Ha, Cho Sungkyu, Kim Eung Re, Kim Yong Jin
Department of Thoracic and Cardiovascular Surgery, Bucheon Sejong General Hospital, Bucheon, South Korea.
Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital, Seoul, South Korea.
J Thorac Dis. 2025 May 30;17(5):2980-2988. doi: 10.21037/jtd-2024-2185. Epub 2025 May 28.
Various surgical techniques have been reported for repairing Ebstein anomaly. Cone repair provides nearly anatomical tricuspid valve (TV) reconstruction with promising outcomes. We reviewed our experience with cone repair to evaluate biventricular remodeling and the outcomes of the annular support procedure.
Between January 2008 and December 2021, cone repair was performed in 33 consecutive patients with Ebstein anomaly. Mean age was 32.0±16.8 years (range, 1.1-66.8 years). Previous TV repair had been performed in two patients with the Hetzer and the Carpentier techniques (6%). Severe tricuspid regurgitation (TR) was observed in 32 patients (97%). Twenty patients had preoperative magnetic resonance imaging (MRI) data; 15 patients had postoperative MRI data.
Modifications included the addition of an annuloplasty band (21 patients with pericardial strips, two patients with prosthetic rings) in 23 patients (69.7%) and papillary muscle repositioning in one patient (3%). Bidirectional cavopulmonary anastomosis was performed in two patients (6%), with one of them undergoing a Fontan operation in the third postoperative years. No mortality was observed. The mean follow-up duration was 7.5±4.6 years. Two patients (6%) required late TV re-repair in the first and sixth postoperative years. At follow-up, five patients (16.1%) reported no or trivial TR, 16 (51.6%) had mild TR, and 5 (16.1%) had mild to moderate TR. Freedom from late TV reoperation was 78.8%±13.4% at 5 years. The TV reoperation rate was significantly low in the patients who underwent tricuspid annuloplasty with a band (P=0.02). Preoperative and postoperative MRI data demonstrated a significant right ventricular (RV) volume decrease after cone repair [RV end-diastolic volume index (mL/m2): preoperative/postoperative =207.4±40.2/105.5±41.3, P=0.001]. Left ventricular ejection fraction (LVEF) remained unchanged after cone repair, while left ventricular stroke volume (LVSV) significantly increased [LVEF (%): preoperative/postoperative =60.8±5.3/61.2±5.4, P=0.10; LVSV (mL): preoperative/postoperative =64.0±1.8/71.4±12.7, P=0.041].
Cone repair for Ebstein anomaly has low mortality and morbidity rates. The addition of an annuloplasty band was associated with a low incidence of the TV reoperation. Moreover, cone repair might impact left ventricular function due to ventricular interdependency. Longer follow-up is essential to determine the late durability of cone repair and both ventricular functional changes.
已有多种手术技术用于修复埃布斯坦畸形。圆锥修复术可实现近乎解剖学的三尖瓣重建，效果良好。我们回顾了我们的圆锥修复术经验，以评估双心室重塑及瓣环支撑手术的效果。
2008年1月至2021年12月期间，连续33例埃布斯坦畸形患者接受了圆锥修复术。平均年龄为32.0±16.8岁（范围1.1 - 66.8岁）。2例患者（6%）曾采用赫策尔和卡彭蒂埃技术进行过三尖瓣修复。32例患者（97%）存在严重三尖瓣反流（TR）。20例患者有术前磁共振成像（MRI）数据；15例患者有术后MRI数据。
改良措施包括23例患者（69.7%）加用瓣环成形带（21例使用心包条带，2例使用人工瓣环），1例患者（3%）进行乳头肌重新定位。2例患者（6%）进行了双向腔肺吻合术，其中1例在术后第3年接受了Fontan手术。无死亡病例。平均随访时间为7.5±4.6年。2例患者（6%）在术后第1年和第6年需要再次进行三尖瓣修复。随访时，5例患者（16.1%）报告无或轻微TR，16例（51.6%）有轻度TR，5例（16.1%）有轻度至中度TR。5年时无需再次进行三尖瓣手术的比例为78.8%±13.4%。使用带瓣环成形术的患者三尖瓣再次手术率显著较低（P = 0.02）。术前和术后MRI数据显示，圆锥修复术后右心室（RV）容积显著减小[RV舒张末期容积指数（mL/m²）：术前/术后 = 207.4±40.2/105.5±41.3，P = 0.001]。圆锥修复术后左心室射血分数（LVEF）保持不变，而左心室每搏输出量（LVSV）显著增加[LVEF（%）：术前/术后 = 60.8±5.3/61.2±5.4，P = 0.10；LVSV（mL）：术前/术后 = 64.0±1.8/71.4±12.7，P = 0.041]。
埃布斯坦畸形的圆锥修复术死亡率和发病率较低。加用瓣环成形带与三尖瓣再次手术发生率低相关。此外，由于心室相互依存性，圆锥修复术可能会影响左心室功能。需要更长时间的随访来确定圆锥修复术的远期耐久性以及双心室功能变化。
