Cardiac Arrhythmia Service, St Vincent's University Hospital, Elm Park, Dublin, Ireland.
Ir J Med Sci. 2011 Dec;180(4):799-805. doi: 10.1007/s11845-011-0744-y. Epub 2011 Aug 7.
Neuromuscular disorders are not among the common causes of cardiomyopathy in the general population; however, cardiomyopathy is known to occur in several neuromuscular disorders including Friedreich's Ataxia (FA). In patients with neuromuscular disorders, concomitant cardiac involvement contributes significantly to morbidity and mortality and often leads to premature death.
An extensive literature search of Medline and Pubmed was conducted to include all published reports on cardiac involvement in FA. Secondary articles were identified from key paper reference listings.
Hypertrophic cardiomyopathy is a cardinal feature of FA; therefore all FA patients should be screened for cardiomyopathy. A cardiac examination, ECG and ECHO are advised at diagnosis, and also on the development of any cardiac symptoms. Treatment is determined by the presence of symptoms, the presence of left ventricular outflow gradient and the sudden death risk. Institution of aggressive medical therapy early in the course of the disease may help improve quality of life and provide survival benefit.
神经肌肉疾病并非一般人群中心肌病的常见病因；然而，几种神经肌肉疾病可导致心肌病，包括弗里德里希共济失调（FA）。在神经肌肉疾病患者中，伴发的心脏受累显著增加发病率和死亡率，并常导致过早死亡。
对 Medline 和 Pubmed 进行广泛的文献检索，以纳入关于 FA 中心脏受累的所有已发表报告。从关键论文的参考文献列表中确定次要文章。
肥厚型心肌病是 FA 的主要特征；因此，所有 FA 患者均应筛查心肌病。建议在诊断时以及出现任何心脏症状时进行心脏检查、心电图和超声心动图检查。治疗取决于症状的存在、左心室流出道梯度的存在和猝死风险。在疾病早期积极进行药物治疗可能有助于提高生活质量并提供生存获益。
