Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London, UK.
Institute of Cardiovascular Sciences, University College London, London, UK.
Arch Dis Child. 2019 May;104(5):471-475. doi: 10.1136/archdischild-2018-315366. Epub 2018 Nov 9.
Children with hypertrophic cardiomyopathy (HCM) have historically been considered to be high-risk candidates for general anaesthesia (GA), but there is currently a paucity of evidence regarding the safety of anaesthesia and perioperative outcomes in this population.
Clinical features and outcomes of all paediatric patients (<18 years) with HCM undergoing GA between 2000 and 2016 were reviewed.
86 patients (median 12.4 years (IQR 6.5, 14.9)) underwent 164 separate GA procedures. Aetiology included non-syndromic disease (n=44, 56%), malformation syndromes (n=22, 26%), inborn error of metabolism (n=10, 12%) and neuromuscular disease (n=4, 5%). At the time of GA, mean maximal wall thickness (MWT) on echocardiography was 19 mm (SD±8 mm), 23 (14%) patients had severe left ventricular hypertrophy (MWT>30 mm) and 35 (21%) patients had a haemodynamically significant left ventricular outflow tract (LVOT) gradient (>50 mm Hg). The majority (n=143, 87%) had no perioperative complications. 20 (12%) patients had minor perioperative complications: bradycardia (n=4), hypotension (n=15) or transient ST segment changes (n=1). One (0.6% of GA procedures) patient experienced a cardiac arrest during anaesthetic induction with death occurring 3 days later. Clinical parameters (including age, MWT, LVOT gradient, systolic and diastolic dysfunction) were not associated with an increased risk of complications CONCLUSIONS: This is the largest published series to date of paediatric patients with HCM undergoing GA, which demonstrates that, in an expert centre, patients can be anaesthetised with a relatively low perianaesthetic mortality (0.6%) and prevalence of minor complications (12%). Future studies are required to systematically identify clinical features that may predict anaesthetic risk.
患有肥厚型心肌病（HCM）的儿童在历史上被认为是全身麻醉（GA）的高风险候选者，但目前关于该人群麻醉安全性和围手术期结果的证据很少。
回顾了 2000 年至 2016 年间接受 GA 的所有患有 HCM 的儿科患者（<18 岁）的临床特征和结局。
86 名患者（中位数 12.4 岁（IQR 6.5，14.9））接受了 164 次单独的 GA 手术。病因包括非综合征疾病（n=44，56%）、畸形综合征（n=22，26%）、先天性代谢错误（n=10，12%）和神经肌肉疾病（n=4，5%）。在 GA 时，超声心动图上的平均最大壁厚度（MWT）为 19 mm（SD±8 mm），23（14%）名患者有严重左心室肥厚（MWT>30 mm），35（21%）名患者有左心室流出道（LVOT）压差>50 mm Hg。大多数（n=143，87%）患者无围手术期并发症。20（12%）名患者有轻微围手术期并发症：心动过缓（n=4）、低血压（n=15）或短暂 ST 段改变（n=1）。1 名（GA 程序的 0.6%）患者在麻醉诱导期间发生心脏骤停，3 天后死亡。临床参数（包括年龄、MWT、LVOT 压差、收缩和舒张功能障碍）与并发症风险增加无关。
这是迄今为止发表的关于接受 GA 的患有 HCM 的儿科患者的最大系列研究，表明在专家中心，患者可以在相对较低的围手术期死亡率（0.6%）和轻微并发症发生率（12%）下接受麻醉。需要进一步的研究来系统地确定可能预测麻醉风险的临床特征。
