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小儿肥厚型心肌病(HCM)为一种单基因疾病,多数呈常染色体显性遗传,主要基因编码在肌节蛋白;小儿HCM的病因和预后与成人不尽相同,除特发性外,可见先天代谢缺陷、各种畸形综合征、神经肌肉疾病等引起的HCM。现阐述小儿HCM的病理和病理生理、临床表现及诊断方法,并介绍药物治疗的进展和Morrow心肌切除术、经皮室间隔消融术、双腔起搏以及植入式心脏复律除颤器的适应证。
Pediatric hypertrophic cardiomyopathy (HCM) is a single gene disease, mostly autosomal dominant inheritance, the main gene is encoded in the sarcomeric protein; pediatric HCM etiology and prognosis are not the same with adults, in addition to idiopathic, can be seen Congenital metabolic defects, various deformities syndrome, neuromuscular disease caused by HCM. The pathology, pathophysiology, clinical presentation and diagnosis of pediatric HCM are described and the progress of pharmacotherapy and Morrow myocardial resection, percutaneous transluminal ablation, dual-chamber pacing and implantable cardioverter defibrillator Indications.