Liu Jia, Przybylski Robert, Mari Mikaela J, Foz Carine, Kobayashi Ryan L, Abrams Dominic, Nasr Viviane G
Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Paediatr Anaesth. 2025 Aug;35(8):649-656. doi: 10.1111/pan.15120. Epub 2025 May 2.
Pediatric cardiomyopathy is rare with an estimated annualized incidence of just over 1 per 100 000 children. However, many of these children have systemic disorders underlying their cardiomyopathy, including RASopathies, muscular dystrophies, and inborn errors of metabolism, and thus may require noncardiac procedural interventions or diagnostic studies. There is a paucity of data about the rate and nature of periprocedural complications and outcomes in this patient population.
This study aims to describe children with cardiomyopathy undergoing procedures or diagnostic studies requiring anesthesia, and their perioperative outcomes.
We performed a single-center retrospective cohort study using each procedural event as the unit of analysis. Perioperative (intraoperative and 72 h postoperatively) data and outcomes were recorded. Logistic regression was used to identify risk factors for adverse events.
We identified 98 patients who underwent a total of 231 procedures. There were 26 total adverse events (11%), including four major adverse events (involving three distinct patients). ASA-PS class 4 status was associated with an increased likelihood of any adverse event, and all the patients who experienced major adverse events had moderate or greater left ventricular dysfunction.
Anesthesia in children with cardiomyopathy is associated with a low (< 2%), but notable, rate of major adverse events. The risk of major adverse events appears to be highest in children with marked left ventricular dysfunction and was often associated with respiratory events in our cohort.
小儿心肌病较为罕见，估计年发病率略高于每10万名儿童中有1例。然而，这些儿童中的许多人在心肌病的基础上存在全身性疾病，包括RASopathies、肌肉萎缩症和先天性代谢缺陷，因此可能需要非心脏程序性干预或诊断性检查。关于这一患者群体围手术期并发症的发生率和性质以及结局的数据很少。
本研究旨在描述接受需要麻醉的程序性干预或诊断性检查的心肌病患儿及其围手术期结局。
我们进行了一项单中心回顾性队列研究，以每个程序性事件作为分析单位。记录围手术期（术中及术后72小时）的数据和结局。采用逻辑回归分析确定不良事件的危险因素。
我们确定了98例患者，共进行了231项程序性干预。总共有26例不良事件（11%），包括4例严重不良事件（涉及3例不同患者）。美国麻醉医师协会身体状况分级（ASA-PS）为4级与发生任何不良事件的可能性增加相关，所有发生严重不良事件的患者均有中度或更严重的左心室功能障碍。
心肌病患儿麻醉相关的严重不良事件发生率较低（<2%），但值得注意。严重不良事件的风险在左心室功能明显受损的患儿中似乎最高，且在我们的队列中常与呼吸事件相关。
