Senni M, Chauvaud S, Crupi G, Procopio A, Bianchi T
Divisione di Cardiochirurgia, Ospedali Riuniti, Bergamo.
G Ital Cardiol. 1996 Dec;26(12):1415-20.
Various surgical techniques have been used for correction of Ebstein's anomaly. This paper reports our early and intermediate term results for repair of this disease using the technique initially employed by Carpentier.
Since December 1987 through December 1991, 13 consecutive patients with Ebstein's anomaly underwent a Carpentier's type of repair. Their mean age was 16.3 years ranging from 6 to 51 years. The indications for operation were advanced NYHA functional class (III-IV) in 4 cases, cyanosis in 4, severe arrhythmia in 3 and cyanosis with arrhythmia in 2. Associated malformations were present in 9 patients. Eight had an atrial septal defect and four of them had, also, mitral prolapse whereas the remaining patient had a ventricular septal defect with subaortic stenosis. Tricuspid regurgitation was severe in 12 cases and moderate in one. According to the anatomo-functional classification described by Carpentier, seven patients had Ebstein's type C, whereas a type B was present in five patients and only one patient had a type A.
There were three hospital deaths all of which occurred in patients with type C anomaly. Causes of death were respectively low output syndrome, sepsis and cerebral hemorrhage. Follow-up ranged from 49 to 105 months (median 58 months). There were no late deaths and all patients were in NYHA functional class I or II. Preoperative arrhythmia was improved or abolished in 4 out of 5 patients. Echocardiography showed that tricuspid regurgitation was absent in 2, mild in 4, moderate in 3 and severe in the remaining patient.
Repair of Ebstein's anomaly, using the technique reported by Carpentier, can be accomplished with an acceptable operative risk and satisfactory intermediate term results. Echocardiography has an important role in the diagnosis of Ebstein's anomaly as well as in the indications and timing for repair and it is particularly useful for intra- and postoperative monitoring and anatomofunctional evaluation.
多种外科技术已被用于矫正埃布斯坦畸形。本文报告了我们采用最初由卡彭蒂埃使用的技术修复该疾病的早期和中期结果。
1987年12月至1991年12月，连续13例埃布斯坦畸形患者接受了卡彭蒂埃式修复。他们的平均年龄为16.3岁，范围从6岁到51岁。手术指征为纽约心脏协会（NYHA）心功能分级晚期（III - IV级）4例，发绀4例，严重心律失常3例，发绀合并心律失常2例。9例患者存在相关畸形。8例有房间隔缺损，其中4例还合并二尖瓣脱垂，其余1例有室间隔缺损合并主动脉瓣下狭窄。三尖瓣反流严重12例，中度1例。根据卡彭蒂埃描述的解剖功能分类，7例患者为C型埃布斯坦畸形，5例为B型，仅1例为A型。
有3例医院死亡，均发生在C型畸形患者中。死亡原因分别为低心排血量综合征、败血症和脑出血。随访时间为49至105个月（中位数58个月）。无晚期死亡，所有患者纽约心脏协会心功能分级为I级或II级。5例患者中有4例术前心律失常得到改善或消除。超声心动图显示，2例无三尖瓣反流，4例轻度反流，3例中度反流，其余1例严重反流。
采用卡彭蒂埃报告的技术修复埃布斯坦畸形，手术风险可接受，中期结果令人满意。超声心动图在埃布斯坦畸形的诊断、修复指征和时机方面具有重要作用，尤其对术中和术后监测及解剖功能评估很有用。
