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白塞病的四重瓣膜置换术:一例报告

Quadruple Valve Replacement for Behçet's Syndrome: A Case Report.

作者信息

Liu Hongyan, Hua Zhengdong, He Bihui, Tao Liang

机构信息

Department of Cardiovascular Surgery, Wuhan Asia Heart Hospital, Wuhan University of Science and Technology, Hubei, China.

出版信息

Catheter Cardiovasc Interv. 2025 Aug;106(2):1051-1055. doi: 10.1002/ccd.31579. Epub 2025 Jun 2.

DOI:10.1002/ccd.31579
PMID:40452468
Abstract

BACKGROUND

Behcet's, a systemic autoimmune vasculitis, often leads to cardiovascular complications sunch as valvular damage and aortic root pseudoaneurysms. Concurrent involvement of all four cardiac valves is exceptionally rare, posing significant surgical challenges due to tissue fraagility, inflammatory destruction, and high reoperation risks.

OBJECTIVE

To describe a tailored surgical strategy for quadruple replacement in a Behcet's syndrome patient with extensive valvular lesions, aortic root pseudoaneurysm, and prior mechanical valve failure, emphasizing techniques to address anatomical complexity and enable future transcatheter interventions.

METHODS

A 51-year-old female with Behcet's syndrome and prior aortic/pulmonary valve replacements underwent reoperation for aortic root pseudoaneurysm, paravalvular leaks, severe mitral/tricuspid regurgitation, and pulmonary valve thrombosis. Key techniques include three parts, firstly, radical debridement of necrotic tissue and annular enlargement (aortic: 20-25mm; mitral: 25-29mm). Second, modified "chimney" Commando-Bentall hybrid procedure with a tongue-shaped vascular path to reconstruct the aorto-mitral curtain and reinforce the aortic root. Third, "Sandwich" technique for aortic root reconstruction to minimize paravalvular leakage.

RESULTS

The surgery was successful, with uneventful recovery and discharge on postoperative day 30. Six-month follow-up confirmed patient survival, stable valve function, and no recurrent leaks. Dual annular enlargement facilitated larger protheses, reducing patient-prothesis mismatch and preserving options for future transcatheter valve im plantation (TAVI).

CONCLUSIONS

This cases highlights the feasibility of combining annular enlargement, root reinforcement, and bioprosthetic valve replacement in Behcet's syndrome with pan-valvular involvement. The hybrid chimney-Bentall technique and meticulous root reconstruction address inflammatory tissue vulnerability while enabling future minimally invasive interventions. This approach optimizes immediate outcomes and long-term durability in complex autoimmune-associated valvulopathies.

摘要

背景

白塞病是一种全身性自身免疫性血管炎,常导致心血管并发症,如瓣膜损害和主动脉根部假性动脉瘤。同时累及所有四个心脏瓣膜极为罕见,由于组织脆弱、炎症破坏和再次手术风险高,给手术带来了重大挑战。

目的

描述一种针对患有广泛瓣膜病变、主动脉根部假性动脉瘤且曾有机械瓣膜故障的白塞病患者进行四联瓣膜置换的定制手术策略,强调应对解剖复杂性并为未来经导管干预创造条件的技术。

方法

一名51岁患有白塞病且曾行主动脉/肺动脉瓣膜置换术的女性因主动脉根部假性动脉瘤、瓣周漏、严重二尖瓣/三尖瓣反流和肺动脉瓣血栓形成接受再次手术。关键技术包括三个部分,首先,彻底清除坏死组织并扩大瓣环(主动脉:20 - 25毫米;二尖瓣:25 - 29毫米)。其次,采用改良的“烟囱”式康曼多 - 本塔尔杂交手术,通过舌形血管路径重建主动脉 - 二尖瓣隔膜并加固主动脉根部。第三,采用“三明治”技术进行主动脉根部重建,以尽量减少瓣周漏。

结果

手术成功,术后恢复顺利,于术后第30天出院。六个月随访证实患者存活,瓣膜功能稳定,无复发性漏血。双重瓣环扩大便于使用更大的人工瓣膜,减少患者与人工瓣膜不匹配,并保留了未来经导管瓣膜植入(TAVI)的选择。

结论

本病例突出了在白塞病全瓣膜受累患者中联合瓣环扩大、根部加固和生物人工瓣膜置换的可行性。杂交烟囱 - 本塔尔技术和细致的根部重建解决了炎症组织的脆弱性问题,同时为未来的微创干预创造了条件。这种方法优化了复杂自身免疫相关瓣膜病的近期疗效和长期耐久性。

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