Abstract:

Hypertrophic cardiomyopathy（HCM） is the second commonest form of heart muscle disease affecting children and adolescents and is a leading cause of sudden death in young athletes. The aetiology of HCM is heterogeneous in the paediatric population，and includes inborn errors of metabolism，neuromuscular disorders and malformation syndromes. However，most cases of apparently idiopathic HCM in childhood are caused by mutations in cardiac sarcomere protein genes. Hypertrophic obstructive cardiomyopathy（HOCM） is not uncommon in children who don’t respond to drug treatment；for such children，surgical treatment is the only solution. Previous routine surgical methods include modified Konno operation，modified extended Morrow operation and interventional alcohol ablation or radiofrequency ablation for pediatric patients. At present， the reports and large-scale clinical experience are rare.

Key words: hypertrophic obstructive cardiomyopathy, surgical treatment, child

摘要：

肥厚型心肌病（HCM）是影响儿童和青少年的第二种常见心肌病，是年轻运动员猝死的主要原因。HCM的病因在儿科人群中包括先天代谢异常、神经肌肉疾病和畸形综合征等。然而，大多数儿童期特发性HCM的病例是由心脏肌节蛋白基因突变引起的。对药物治疗无效的肥厚型梗阻性心肌病（HOCM）患儿并不少见；对于此类患儿，手术治疗往往是惟一解决方案。既往常规的外科手术方式包括改良Konno手术，改良扩大Morrow手术，对于儿科患儿行介入酒精消融或射频消融的手术治疗，目前鲜有相关报道和大规模的临床经验。

关键词: 肥厚型梗阻型心肌病, 外科治疗, 儿童