Postoperative Outcomes and Valvular Involvement in Loeys-Dietz Syndrome: A Multicenter Cohort Study of Surgical Risk and Complications

IF 2.1 3区医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS

American Journal of Cardiology Pub Date : 2025-08-21 DOI:10.1016/j.amjcard.2025.08.032

Hussein Abdul Nabi MD , Sant Kumar MD , Luke Dreher MD , George Bcharah BS , Nour Odeh MBBS , Mayar Alatout MBBS , Yuxiang Wang MD , Mayowa A. Osundiji MBBS, PhD , Bryan Barrus MD , Kristen Sell-Dotin MD , Chadi Ayoub MD, PhD , Hicham El Masry MD , Fadi E. Shamoun MD

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引用次数: 0

Abstract

Loeys-Dietz syndrome (LDS) is a connective tissue disorder characterized by significant vascular and valvular abnormalities; however, perioperative outcomes involving aortic and valvular interventions remain underreported. This study aimed to evaluate surgical interventions and postoperative complications in LDS patients. We retrospectively reviewed 94 patients diagnosed with LDS from 2018 to 2024 across Mayo Clinic sites. Data on surgical procedures, postoperative complications, echocardiographic findings, and arrhythmic events were collected. Valve replacements, valve types, and anticoagulation regimens were analyzed. Categorical variables were reported as frequencies and percentages. Ascending aortic aneurysms were present in 77 patients (81.9%), mitral valve prolapse in 33 (35.1%), mitral regurgitation in 36 (38.3%), and tricuspid valve regurgitation in 37 (39.4%). A total of 61 patients (64.9%) underwent at least 1 cardiovascular surgical procedure, most commonly aortic root surgery in 59 patients (62.8%) and aortic valve repair/replacement in 39 patients (41.5%). The median follow-up period from the first surgery to the last follow-up was 6.1 years (IQR = 9.2 years). Major postoperative complications occurred in 32 patients (52.5%), including severe bleeding in 15 patients (24.6%) and neurological events in 6 patients (9.8%). New-onset arrhythmias were documented in 25 patients (41.0%), primarily atrial fibrillation/flutter in 12 patients (19.7%). Among patients with mechanical valves, 21 of 28 (75%) on warfarin experienced bleeding complications. In conclusion, this study highlights the high burden of both aortic and valvular surgical interventions in LDS patients and the associated postoperative risks, reflecting underlying connective tissue fragility. Tailored perioperative strategies are needed, and future studies incorporating LDS genotyping may improve risk stratification and management.

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Loeys-Dietz综合征的术后结局和瓣膜受累：手术风险和并发症的多中心队列研究

Loeys-Dietz综合征（LDS）是一种结缔组织疾病，其特征是明显的血管和瓣膜异常；然而，涉及主动脉瓣和瓣膜干预的围手术期结果仍未得到充分报道。本研究旨在评估LDS患者的手术干预和术后并发症。我们回顾性分析了梅奥诊所2018年至2024年诊断为LDS的94例患者。收集了手术过程、术后并发症、超声心动图结果和心律失常事件的数据。分析瓣膜置换术、瓣膜类型和抗凝治疗方案。分类变量以频率和百分比报告。升主动脉瘤77例（81.9%），二尖瓣脱垂33例（35.1%），二尖瓣反流36例（38.3%），三尖瓣反流37例（39.4%）。共有61例患者（64.9%）接受了至少一次心血管手术，最常见的是59例（62.8%）主动脉根部手术，39例（41.5%）主动脉瓣修复/置换术。第一次手术至最后一次随访的中位随访时间为6.1年（IQR = 9.2年）。术后发生重大并发症32例（52.5%），其中大出血15例（24.6%），神经系统事件6例（9.8%）。新发心律失常25例（41.0%），主要为房颤/扑动12例（19.7%）。在28例使用华法林的机械瓣膜患者中，有21例（75%）出现出血并发症。总之，本研究强调了LDS患者主动脉和瓣膜手术干预的高负担和相关的术后风险，反映了潜在的结缔组织脆弱性。需要量身定制围手术期策略，未来纳入LDS基因分型的研究可能会改善风险分层和管理。

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来源期刊

American Journal of Cardiology 医学-心血管系统

CiteScore

4.00

自引率

3.60%

发文量

698

审稿时长

33 days

期刊介绍： Published 24 times a year, The American Journal of Cardiology® is an independent journal designed for cardiovascular disease specialists and internists with a subspecialty in cardiology throughout the world. AJC is an independent, scientific, peer-reviewed journal of original articles that focus on the practical, clinical approach to the diagnosis and treatment of cardiovascular disease. AJC has one of the fastest acceptance to publication times in Cardiology. Features report on systemic hypertension, methodology, drugs, pacing, arrhythmia, preventive cardiology, congestive heart failure, valvular heart disease, congenital heart disease, and cardiomyopathy. Also included are editorials, readers'' comments, and symposia.