Department of Cardiovascular Surgery, Fuwai Hospital, National Center for Cardiovascular Diseases, 571193Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Asian Cardiovasc Thorac Ann. 2022 Jan;30(1):92-97. doi: 10.1177/02184923211041285. Epub 2021 Sep 27.
Hypertrophic cardiomyopathy (HCM) characterized by asymmetric ventricular septal hypertrophy, is the commonest cause of sudden cardiac death (SCD) in the young. The underlying etiology of HCM in the childhood and adolescent patients is diverse. Moreover, the prognosis of pediatric HCM depends on the age of presentation and etiology. Despite the complexity of children with obstructive HCM, surgical treatment results in a favorable outcome for carefully selected patients in experienced tertiary referral center in contemporary era. Implantable cardioverter-defibrillator (ICD) remains the most effective and reliable treatment to prevent SCD. New pediatric SCD risk prediction model, which has good discrimination and calibration and can distinguish patients who are most benefit from an ICD implantation, is expected to be further refined in the future.
肥厚型心肌病(HCM)以心室间隔不对称性肥厚为特征,是年轻人发生心源性猝死(SCD)的最常见原因。儿童和青少年患者的 HCM 病因多种多样。此外,儿科 HCM 的预后取决于发病年龄和病因。尽管梗阻性 HCM 患儿情况复杂,但在当代,在经验丰富的三级转诊中心,对精心挑选的患者进行手术治疗可获得良好的效果。植入式心脏复律除颤器(ICD)仍然是预防 SCD 最有效的可靠治疗方法。新的儿科 SCD 风险预测模型具有良好的区分度和校准度,可以区分最受益于 ICD 植入的患者,预计未来将进一步完善。