From the Department of Anesthesiology and Critical Care Medicine, Johns Hopkins Hospital, Baltimore, Maryland.
Anesth Analg. 2015 Mar;120(3):554-569. doi: 10.1213/ANE.0000000000000538.
Hypertrophic cardiomyopathy (HCM) is a relatively common disorder that anesthesiologists encounter among patients in the perioperative period. Fifty years ago, HCM was thought to be an obscure disease. Today, however, our understanding and ability to diagnose patients with HCM have improved dramatically. Patients with HCM have genotypic and phenotypic variability. Indeed, a subgroup of these patients exhibits the HCM genotype but not the phenotype (left ventricular hypertrophy). There are a number of treatment modalities for these patients, including pharmacotherapy to control symptoms, implantable cardiac defibrillators to manage malignant arrhythmias, and surgical myectomy and septal ablation to decrease the left ventricular outflow obstruction. Accurate diagnosis is vital for the perioperative management of these patients. Diagnosis is most often made using echocardiographic assessment of left ventricular hypertrophy, left ventricular outflow tract gradients, systolic and diastolic function, and mitral valve anatomy and function. Cardiac magnetic resonance imaging also has a diagnostic role by determining the extent and location of left ventricular hypertrophy and the anatomic abnormalities of the mitral valve and papillary muscles. In this review on hypertrophic cardiomyopathy for the noncardiac anesthesiologist, we discuss the clinical presentation and genetic mutations associated with HCM, the critical role of echocardiography in the diagnosis and the assessment of surgical interventions, and the perioperative management of patients with HCM undergoing noncardiac surgery and management of the parturient with HCM.
肥厚型心肌病（HCM）是一种相对常见的疾病，麻醉医生在围手术期会遇到此类患者。五十年前，HCM 被认为是一种罕见疾病。然而，如今我们对 HCM 患者的认识和诊断能力已经有了显著提高。HCM 患者存在基因型和表型的可变性。实际上，这些患者中的一部分表现出 HCM 基因型但没有表型（左心室肥厚）。这些患者有多种治疗方法，包括药物治疗以控制症状、植入式心脏除颤器以治疗恶性心律失常，以及外科心肌切除术和室间隔消融术以减少左心室流出道梗阻。准确的诊断对这些患者的围手术期管理至关重要。诊断通常通过超声心动图评估左心室肥厚、左心室流出道梯度、收缩和舒张功能以及二尖瓣解剖结构和功能来进行。心脏磁共振成像也通过确定左心室肥厚的程度和位置以及二尖瓣和乳头肌的解剖异常来发挥诊断作用。在这篇非心脏麻醉医生的肥厚型心肌病综述中，我们讨论了 HCM 的临床表现和基因突变、超声心动图在诊断和评估手术干预中的关键作用，以及非心脏手术患者和 HCM 产妇的围手术期管理。
