Department of Cardiology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, China.
BMC Cardiovasc Disord. 2023 Jan 31;23(1):57. doi: 10.1186/s12872-023-03057-6.
Anti-mitochondrial antibody (AMA)-positive inflammatory myopathy, a rare type of idiopathic inflammatory myopathy which was frequently difficult to diagnose, can affect muscles and the structure and electrical conduction of the heart. Early identification and treatment of this myopathy can prevent serious cardiovascular adverse events and improve cardiac function.
We report a patient who experienced repeated syncope, ventricular tachycardia (VT) and heart failure accompanied by weakness and muscle atrophy. He was initially diagnosed with dilated cardiomyopathy and received implantable cardioverter-defibrillator therapy. He was subsequently misdiagnosed as muscular dystrophy due to progressive muscular atrophy. However, the patient developed repeated and refractory VT storms that were not alleviated by conventional therapy. Finally, he was diagnosed with AMA-positive inflammatory myopathy with cardiac injuries. The patient was markedly recovered by being treated with immunosuppressive and immunomodulatory therapy.
AMA could be screened when discovering myopathies accompanied by unexplained cardiac symptoms. Our findings provide insights into the diagnosis and therapy of this rare and severe disease.
抗线粒体抗体(AMA)阳性炎性肌病是一种罕见的特发性炎性肌病,常难以诊断,可影响肌肉以及心脏的结构和电传导。早期识别和治疗这种肌病可以预防严重的心血管不良事件并改善心功能。
我们报告了一例患者,他经历了反复晕厥、室性心动过速(VT)和心力衰竭,伴有乏力和肌肉萎缩。他最初被诊断为扩张型心肌病,并接受了植入式心脏复律除颤器治疗。随后,由于进行性肌肉萎缩,他被误诊为肌肉营养不良症。然而,患者反复出现且难以控制的 VT 风暴,常规治疗无法缓解。最终,他被诊断为伴有心脏损伤的 AMA 阳性炎性肌病。该患者经免疫抑制和免疫调节治疗后明显恢复。
当发现伴有不明原因心脏症状的肌病时,可筛查 AMA。我们的发现为这种罕见且严重的疾病的诊断和治疗提供了思路。