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马凡综合征患者行保留主动脉瓣的主动脉根部替换术(David I 手术):20 年来单中心 100 多例患者的经验†。

Valve-sparing aortic root replacement (David I procedure) in Marfan disease: single-centre 20-year experience in more than 100 patients†.

机构信息

Department of Cardiothoracic, Transplantation and Vascular Surgery, Hannover Medical School, Hannover, Germany.

出版信息

Eur J Cardiothorac Surg. 2019 Mar 1;55(3):476-483. doi: 10.1093/ejcts/ezy300.

DOI:10.1093/ejcts/ezy300
PMID:30169770
Abstract

OBJECTIVES

Valve-sparing aortic root replacement (VSARR) is recommended for patients with aortic root dilatation and preserved aortic valve cusp morphology. The durability of VSARR in Marfan patients has been questioned. The aim of our study was to establish the long-term outcomes of VSARR in Marfan patients.

METHODS

Between 1993 and 2015, 582 patients underwent VSARR (David I reimplantation) at our institution. Of these patients, 104 had Marfan disease. Thirteen surgeons performed the procedures in this group. The mean follow-up time was 12 ± 5.4 years (1201 patient-years).

RESULTS

Early mortality was 0.96%, and long-term survival was 91% at 10 years and 76% at 20 years in Marfan patients. Marfan patients had a significantly better survival compared to non-Marfan patients (P < 0.0001). Freedom from aortic-valve reoperation was 86% at 10 years and 80% at 20 years in Marfan patients. The reoperation rate was similar in Marfan and non-Marfan patients (P = 0.60). Morphological perioperative features (untreated prolapse, commissural plasty, cusp plasty and graft size mismatch) predicted long-term mortality (P = 0.0054). Graft size mismatch and untreated prolapse predicted structural valve deterioration (both P < 0.0001). Long-term valve function in event-free survivors was excellent [mean gradient 4.2 (2.9-6.9), 98% aortic regurgitation ≤ mild]. There were no valve-related thromboembolic or bleeding events. The endocarditis rate was 0.96%. Only 17% of the patients were on oral anticoagulants during the follow-up.

CONCLUSIONS

VSARR using the David I reimplantation technique results in excellent long-term outcomes in Marfan patients. We present the longest follow-up period so far. The genetic disease does not affect long-term valve function. The durability of the repair is affected by morphological perioperative criteria depending on surgical expertise, and dedicated training is recommended.

摘要

目的

对于主动脉根部扩张且主动脉瓣瓣叶形态保存的患者,推荐行保留主动脉瓣的主动脉根部替换术(VSARR)。马凡综合征患者行 VSARR 的耐久性曾受到质疑。本研究旨在评估 VSARR 在马凡综合征患者中的长期疗效。

方法

1993 年至 2015 年,我院共对 582 例患者行 VSARR(David I 重植入术),其中 104 例为马凡综合征患者。该组患者由 13 位外科医生进行手术。平均随访时间为 12±5.4 年(1201 患者年)。

结果

早期死亡率为 0.96%,马凡综合征患者的 10 年和 20 年生存率分别为 91%和 76%。马凡综合征患者的生存率显著优于非马凡综合征患者(P<0.0001)。马凡综合征患者的主动脉瓣再手术率 10 年时为 86%,20 年时为 80%。马凡综合征和非马凡综合征患者的再手术率相似(P=0.60)。围手术期形态学特征(未治疗的瓣叶脱垂、交界区成形术、瓣叶成形术和移植物大小不匹配)预测长期死亡率(P=0.0054)。移植物大小不匹配和未治疗的瓣叶脱垂预测结构瓣叶退行性变(均 P<0.0001)。无事件生存者的长期瓣膜功能良好[平均跨瓣梯度 4.2(2.9-6.9),98%主动脉瓣反流≤轻度]。无瓣膜相关血栓栓塞或出血事件。心内膜炎发生率为 0.96%。随访期间,仅 17%的患者服用口服抗凝剂。

结论

采用 David I 重植入技术行 VSARR 可使马凡综合征患者获得良好的长期疗效。本研究报道了迄今为止最长的随访时间。该遗传性疾病不影响长期瓣膜功能。修复的耐久性取决于手术专业知识的围手术期形态学标准,建议进行专门培训。

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