埃布斯坦畸形的手术策略:一家儿科医院 28 年的经验。

Mariela Mouratian, Claudia Villalba, Agustina Ramos, Fernando Diez-Mori, M ª Victoria Lafuente, Erica Stelmaszewski, Carla Pasinato, Juliana Medina, Marisa Di Santo, Pablo García-Delucis
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引用次数: 0

摘要

目的:埃布斯坦氏畸形是一种先天性缺陷,其特征是三尖瓣缺乏分层和顶端移位、三尖瓣关闭不全、右心房增大和心室功能障碍。为了分析不同手术策略的结果和演变,我们收集了1990-2018年间接受手术的45名患者的数据。纳入的 26 名患者初次手术的中位年龄为 11.3 岁(范围:13 天-18.6 岁):方法:10 名患者接受了整形手术(38%),11 名患者接受了锥体技术重建手术(42%),5 名患者接受了置换手术(19%)。7名患者需要进行额外干预:腔肺吻合术4人,Cox-maze术4人。9名患者(34.6%)因严重三尖瓣关闭不全而需要再次手术。其中六人曾接受过整形手术,两人接受过生物修复术,一人接受过锥切术:总死亡率为 11.5%(3 例),中位数为术后 10.1 年(范围:5.7-10.12),与心律失常(p = 0.05)、右室(p = 0.008)、左室(p = 0.0001)功能障碍和再次手术(p = 0.03)有关。没有人曾接受过锥切手术。中位随访时间为 6.5 年(范围:1-29.1 年)。91.66%的患者功能分级为I/II级,79.2%为窦性心律:结论:不同经典技术的效果相似,但并非没有并发症和再手术。锥体重建被证明是有效的,手术死亡率低,再次手术的需求较少,中期效果持久。
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Surgical strategies in Ebstein anomaly: 28 years' experience in a pediatric hospital.

Objectives: Ebstein's anomaly is a congenital defect characterized by a lack of delamination and apical displacement of the tricuspid valve, tricuspid insufficiency, right atrial enlargement, and ventricular dysfunction. To analyze the results and evolution of the different surgical strategies, data were collected from 45 patients operated on during 1990-2018. Twenty-six patients were included with a median age at initial surgery of 11.3 years (range: 13 days-18.6 years).

Method: Procedures were plastic 10 patients (38%), cone technique reconstruction 11 (42%), and replacement 5 (19%). Additional interventions were required in 7 patients: cavo-pulmonary anastomosis 4 and Cox-maze 4. Nine patients (34.6%) required reoperation due to severe tricuspid insufficiency. Six had previous plastic, two bioprothesis, and one, cone.

Results: Overall mortality was 11.5% (3) at a median of 10.1 years post-surgery (range: 5.7-10.12) associated with arrhythmias (p = 0.05), right (p = 0.008), left (p = 0.0001) ventricular dysfunction and reoperations (p = 0.03). None were previous conus. Median follow-up was 6.5 years (range: 1-29.1). Ninety-one-point-six percent were in functional class I/II and 79.2% in sinus rhythm.

Conclusions: The results of the different classic techniques were similar, although not free of complications and reoperations. Cone reconstruction proved to be effective, with low surgical mortality, less need for reoperations, and durability in the medium term.

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