Orr William B, Fabi Marianna, Khoury Michael, Dallaire Frederic, Thacker Deepika, Elias Matthew D, Choueiter Nadine F, Lang Sean M, Dahdah Nagib, Harahsheh Ashraf S, Nowlen Todd T, Alsalehi Mahmoud, Misra Nilanjana, Dionne Audrey, Dancey Paul, Lee Simon, Raghuveer Geetha, Norozi Kambiz, Szmuszkovicz Jacqueline R, Mondal Tapas, Hicar Mark D, Khare Manaswitha, Tierney Seda, Portman Michael A, Wehrmann Melissa, Grcic Michelle, Sundaram Balasubramanian, Ganzoury Mona El, Prasad Deepa, Harris Tyler H, Caro-Barri Ana, Garrido-Garcia Luis Martin, Braunlin Elizabeth, Mauriello Daniel, McHugh Kimberly E, McCrindle Brian W
Division of Pediatric Cardiology, Department of Pediatrics, Washington University School of Medicine, St Louis, MO, USA.
Pediatric Emergency Unit, IRCCS Azienda Ospedaliero Universitaria Di Bologna, Bologna, Italy.
Pediatr Cardiol. 2025 May 12. doi: 10.1007/s00246-025-03888-4.
Non-coronary artery systemic arterial aneurysms (SAAs) are rare and an under-reported sequelae of Kawasaki disease (KD). We hypothesize that practices regarding SAA screening and management vary widely among experts and published literature. A survey was sent to members of the International KD Registry regarding their experiences and practices with SAAs in KD patients. For comparison, a systematic scoping review was conducted using PRISMA methodology, from which 25 reports with 83 patients were included. Results from each were compared. Surveys were completed by 48 (56%) of 86 IKDR investigators; 35 (73%) respondents had > 10 years of experience caring for KD patients. However, 33% of respondents had not cared for a patient with SAA. Features prompting assessment for SAA included demographics, presence and degree of coronary artery (CA) involvement, and clinical features, including prolonged/persistent fever, progressing/persistent elevation of inflammatory markers, and resistance to standard treatment. Features prompting screening were somewhat concordant with the characteristics of patients with SAA identified in the scoping review. From the survey, the initial preferred assessment included computed tomographic angiography (48%), ultrasound (29%), and magnetic resonance imaging (24%). In contrast, assessment of patients with SAA from the scoping review commonly used multiple imaging modalities. SAA often regressed, but associated complications included thrombosis, calcification, stenosis, occlusion, and collateral formation. While SAA is a known but rare complication of acute KD, there remains a gap in evidence regarding which patients are at risk, best practices for screening and management, and outcomes. Prospective cohort studies are needed.
非冠状动脉性系统性动脉瘤（SAAs）较为罕见，是川崎病（KD）报告不足的后遗症。我们推测，专家和已发表文献中关于SAA筛查和管理的做法差异很大。我们向国际KD注册中心的成员发送了一份调查问卷，询问他们在KD患者中处理SAA的经验和做法。为作比较，我们采用PRISMA方法进行了系统的范围综述，纳入了25篇报告中的83例患者。对两者的结果进行了比较。86名IKDR研究人员中有48名（56%）完成了调查；35名（73%）受访者有超过10年照顾KD患者的经验。然而，33%的受访者未曾照料过SAA患者。促使对SAA进行评估的特征包括人口统计学特征、冠状动脉（CA）受累情况及程度，以及临床特征，包括持续发热、炎症标志物持续升高、对标准治疗耐药。促使进行筛查的特征与范围综述中确定的SAA患者特征有些一致。从调查来看，最初首选的评估方法包括计算机断层血管造影（48%）、超声（29%）和磁共振成像（24%）。相比之下，范围综述中对SAA患者进行评估时常用多种成像方式。SAA常可消退，但相关并发症包括血栓形成钙化、狭窄、闭塞和侧支形成。虽然SAA是急性KD已知但罕见的并发症，但在哪些患者有风险、筛查和管理的最佳做法以及预后方面，证据仍存在差距。需要进行前瞻性队列研究。
