Anderson Heather N, Dearani Joseph A, Said Sameh M, Norris Mark D, Pundi Kavitha N, Miller Angela R, Cetta Michael L, Eidem Benjamin W, O'Leary Patrick W, Cetta Frank
Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minn, USA.
Congenit Heart Dis. 2014 May-Jun;9(3):266-71. doi: 10.1111/chd.12155. Epub 2013 Dec 23.
Valve repair for pediatric patients with Ebstein anomaly has historically yielded varied results. The cone reconstruction (CR) first described by Da Silva has revolutionized the surgical approach to these patients. This study reports our recent experience with CR in children and young adults with Ebstein anomaly.
Electronic medical records were reviewed for all patients < 21 years old who had surgery to repair Ebstein anomaly at Mayo Clinic Rochester between June 2007 and December 2012. Clinical data including preoperative demographics, intraoperative procedures, and postoperative outcomes were recorded.
Eighty-four patients initially had a cone reconstruction (54% male, mean age 10.1 ± 5.9 years). Indications for operation included cardiomegaly (42%), cyanosis (19%), and heart failure (19%). The preoperative echocardiogram demonstrated severe tricuspid regurgitation in 91% of patients. There was one early death and 3 early CR breakdowns requiring reoperation (2 re-repair, 1 tricuspid replacement). Eighty-two patients (98%) had successful CR at the time of hospital discharge. Patient age, gender, cardiopulmonary bypass time, and aortic cross-clamp time were not associated with early CR failure. Use of a partial or eccentric annuloplasty ring correlated with successful initial CR (P = .01). There have been no early CR breakdowns since 2010. Follow-up information was available for 77 patients (longest follow-up 6.5 years; mean 0.8 ± 0.2 years). The most recent postoperative echocardiogram demonstrated mild or no tricuspid regurgitation in 83%. Tricuspid stenosis (mean gradient > 5 mm Hg) was present in 6 patients. There was one late death (motor vehicle accident) and one late re-repair of the tricuspid valve 4 years after initial operation.
CR in children and young adults with Ebstein anomaly can be performed with low early mortality and excellent durability at short-term follow-up. CR represents an important surgical option for young patients. It is applicable to patients with a broad range of anatomic variability and precludes valve replacement in the vast majority. CR should be considered prior to the deleterious effects of chronic right ventricular volume overload and the development of systolic dysfunction, which hamper long-term prognosis. Therefore, early referral for surgical evaluation is recommended.
对于患有埃布斯坦畸形的儿科患者，瓣膜修复术历来效果各异。达席尔瓦首次描述的圆锥重建术（CR）彻底改变了针对这些患者的手术方法。本研究报告了我们近期在患有埃布斯坦畸形的儿童和青年成人中应用CR的经验。
回顾了2007年6月至2012年12月期间在罗切斯特梅奥诊所接受埃布斯坦畸形修复手术的所有21岁以下患者的电子病历。记录了包括术前人口统计学资料、术中操作和术后结果在内的临床数据。
84例患者最初接受了圆锥重建术（54%为男性，平均年龄10.1±5.9岁）。手术指征包括心脏扩大（42%）、紫绀（19%）和心力衰竭（19%）。术前超声心动图显示91%的患者存在严重三尖瓣反流。有1例早期死亡和3例早期CR失败需要再次手术（2例再次修复，1例三尖瓣置换）。82例患者（98%）在出院时CR成功。患者年龄、性别、体外循环时间和主动脉阻断时间与早期CR失败无关。使用部分或偏心瓣环成形环与初始CR成功相关（P = 0.01）。自2010年以来没有早期CR失败病例。77例患者有随访信息（最长随访6.5年；平均0.8±0.2年）。最近的术后超声心动图显示83%的患者存在轻度或无三尖瓣反流。6例患者存在三尖瓣狭窄（平均压差>5 mmHg）。有1例晚期死亡（机动车事故）和1例在初次手术后4年进行的三尖瓣晚期再次修复。
在患有埃布斯坦畸形的儿童和青年成人中进行CR，早期死亡率低，短期随访耐久性良好。CR是年轻患者的重要手术选择。它适用于解剖变异范围广泛的患者，并且在绝大多数情况下可避免瓣膜置换。在慢性右心室容量超负荷的有害影响和收缩功能障碍发展之前应考虑CR，因为这些会妨碍长期预后。因此，建议早期转诊进行手术评估。
