BACKGROUND Once surgical management is indicated, variation of Ebstein valve morphology affects surgical strategy. This study explored practical, easily measureable, cardiovascular magnetic resonance (CMR)-derived attributes that may contribute to the complexity and risk of cone reconstruction. METHODS A retrospective assessment was performed of Ebstein anomaly patients older than 12 years age, with pre-operative CMR, undergoing cone surgical reconstruction by one surgeon. In addition to clinical data, the CMR-derived Ebstein valve rotation angle (EVRA), area ratios of chamber size, indexed functional RV (RVEDVi) and left ventricular (LV) volumes, tricuspid valve regurgitant fraction (TR%) and other valve attributes were related to early surgical outcome; including death, significant residual TR% or breakdown of repair. RESULTS Of 26 operated patients older than 12 years age, since program start, 20 had pre-op CMR and underwent surgery at median (range) age 20 (14-57) years. TR% was improved in all patients. Four of the 20 CMR patients (20%) experienced early surgical dehiscence of the paravalve tissue, with cone-shaped tricuspid valve intact; one of whom died. A larger EVRA correlated with Carpentier category and was significantly related to dehiscence. If EVRA >60o, relative risk of dehiscence was 3.2 (CI 1.3-4.9, p = 0.03). Those with dehiscence had thickened, more tethered anterior leaflet edges (RR 17, CI 3-100, p < 0.01), smaller pre-operative functional RVEDVi; (132 vs 177 mL/m2, p = 0.04), and were older (median 38 vs 19 years, p = 0.01). TR %, chamber area ratios and LV parameters were not different. CONCLUSIONS Comprehensive CMR assessment characterizes patients prior to cone surgical reconstruction of Ebstein anomaly. Pragmatic observation of larger EVRA, smaller RVEDVi and leaflet thickening, suggests risk of repair tension and dehiscence, and may require specific modification of cone surgical technique, such as leaflet augmentation.

中文翻译：

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通过心血管磁共振对 Ebstein 瓣膜旋转程度进行简单测量，为手术锥体重建的可行性提供了实用指南。


背景 一旦需要进行手术治疗，Ebstein 瓣膜形态的变化就会影响手术策略。这项研究探索了实用的、易于测量的、心血管磁共振 (CMR) 衍生的属性，这些属性可能会导致锥体重建的复杂性和风险。方法对 12 岁以上、术前具有 CMR、由一名外科医生进行锥体手术重建的 Ebstein 异常患者进行回顾性评估。除临床数据外，CMR 衍生的 Ebstein 瓣膜旋转角 (EVRA)、腔室大小面积比、索引功能性 RV (RVEDVi) 和左心室 (LV) 容积、三尖瓣反流分数 (TR%) 和其他瓣膜属性与早期手术结果有关；包括死亡、显着残留 TR% 或修复故障。结果 自项目启动以来，在 26 名年龄超过 12 岁的手术患者中，有 20 名进行了术前 CMR，并在中位年龄（范围）20（14-57）岁接受了手术。所有患者的 TR% 均得到改善。 20 名 CMR 患者中有 4 名 (20%) 经历了瓣周组织的早期手术裂开，但锥形三尖瓣完好无损；其中一人死亡。较大的 EVRA 与 Carpentier 类别相关，并且与开裂显着相关。如果 EVRA >60o，开裂的相对风险为 3.2（CI 1.3-4.9，p = 0.03）。那些裂开的患者前叶边缘增厚、栓系较多（RR 17，CI 3-100，p < 0.01），术前功能性 RVEDVi 较小； （132 与 177 mL/m2，p = 0.04），并且年龄较大（中位数 38 岁与 19 岁，p = 0.01）。 TR %、室面积比和 LV 参数没有不同。结论 在 Ebstein 异常锥体手术重建之前，综合 CMR 评估描述了患者的特征。 对较大 EVRA、较小 RVEDVi 和小叶增厚的实际观察表明存在修复张力和裂开的风险，并且可能需要对锥体手术技术进行特定修改，例如小叶增大。