进行性假肥大性肌营养不良心肌病的诊疗进展

谷洁, 刘宇航, 洪思琦

中国当代儿科杂志 ›› 2026, Vol. 28 ›› Issue (10) : 1289-1297.

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中国当代儿科杂志 ›› 2026, Vol. 28 ›› Issue (10) : 1289-1297. DOI: 10.7499/j.issn.1008-8830.2606154
综述

进行性假肥大性肌营养不良心肌病的诊疗进展

作者信息 +

Duchenne muscular dystrophy-associated cardiomyopathy: advances in diagnosis and management

Author information +
文章历史 +

摘要

进行性假肥大性肌营养不良(Duchenne muscular dystrophy, DMD)是一种X连锁隐性遗传的神经肌肉病,心肌受累往往较为隐匿,心力衰竭已成为影响患者预后的主要因素之一。心脏磁共振成像、斑点追踪超声心动图等新技术为精准评估DMD心肌病提供了依据。目前,糖皮质激素治疗和心血管药物治疗是DMD心肌病的基本治疗手段,基因疗法、细胞治疗、分子靶向药物等也在不断发展。该文对DMD心肌病的致病机制、诊断技术及治疗策略进行综述,旨在为规范临床管理、改善患者生存质量提供参考。

Abstract

Duchenne muscular dystrophy (DMD) is an X-linked recessive neuromuscular disorder in which cardiac involvement is relatively insidious, and heart failure has become one of the major determinants of prognosis. Cardiac magnetic resonance imaging and speckle-tracking echocardiography provide a basis for the precise assessment of DMD-associated cardiomyopathy. At present, glucocorticoid therapy and cardiovascular drug therapy are the basic treatment approaches, and gene therapy, cell therapy, and molecularly targeted drugs are continually evolving. This review summarizes pathogenic mechanisms, diagnostic techniques, and therapeutic strategies for DMD-associated cardiomyopathy, with the aim of informing standardized clinical management and improving patient quality of life.

关键词

进行性假肥大性肌营养不良 / 心肌病 / 心脏磁共振成像 / 心血管药物

Key words

Duchenne muscular dystrophy / Cardiomyopathy / Cardiac magnetic resonance imaging / Cardiovascular agent

引用本文

导出引用
谷洁, 刘宇航, 洪思琦. 进行性假肥大性肌营养不良心肌病的诊疗进展[J]. 中国当代儿科杂志. 2026, 28(10): 1289-1297 https://doi.org/10.7499/j.issn.1008-8830.2606154
Jie GU, Yu-Hang LIU, Si-Qi HONG. Duchenne muscular dystrophy-associated cardiomyopathy: advances in diagnosis and management[J]. Chinese Journal of Contemporary Pediatrics. 2026, 28(10): 1289-1297 https://doi.org/10.7499/j.issn.1008-8830.2606154

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