Tashima Koichiro, Hayashi Masakiyo, Oyoshi Takafumi, Uemura Jo, Korematsu Shinnosuke, Hirata Naoyuki
Department of Anesthesiology, Kumamoto University Hospital, 1-1-1, Honjo, Chuo-Ku, Kumamoto, 860-8556, Japan.
JA Clin Rep. 2024 Aug 8;10(1):49. doi: 10.1186/s40981-024-00734-z.
Mitochondrial cardiomyopathy occurs when impaired mitochondrial energy production leads to myocardial dysfunction. Anesthetic management in such cases is challenging due to risks of circulatory depression associated with anesthesia and mitochondrial dysfunction induced by anesthetics. Although there are reports of anesthetic management for patients with mitochondrial diseases, there are few reports specifically addressing cardiac anesthesia for patients with mitochondrial cardiomyopathy. We present a case where percutaneous mitral valve repair with MitraClip™ was successfully performed under remimazolam anesthesia in a patient with mitochondrial cardiomyopathy who developed functional mitral valve regurgitation due to low cardiac function and cardiomegaly.
A 57-year-old woman was diagnosed with chronic cardiac failure, with a 10-year history of dilated cardiomyopathy. She was diagnosed with mitochondrial cardiomyopathy 8 years ago. Over the past 2 years, her cardiac failure worsened, and mitral valve regurgitation gradually developed. Surgical intervention was considered but deemed too risky due to her low cardiac function, with an ejection fraction of 26%. Therefore, percutaneous MitraClip™ implantation was selected. After securing radial artery and central venous catheterization under sedation with dexmedetomidine, anesthesia was induced with a low dose of remimazolam 4 mg/kg/h. Anesthesia was maintained with remimazolam 0.35-1.0 mg/kg/h and remifentanil 0.1 μg/kg/min. Noradrenaline and dobutamine were administered intraoperatively, and the procedure was completed successfully without circulatory collapse. The patient recovered smoothly from anesthesia and experienced no complications. She was discharged on the eighth day after surgery.
Anesthesia management with remimazolam appears to be a safe and effective for MitraClip™ implantation in patients with mitochondrial cardiomyopathy.
当线粒体能量产生受损导致心肌功能障碍时，会发生线粒体心肌病。由于与麻醉相关的循环抑制风险以及麻醉药引起的线粒体功能障碍，此类病例的麻醉管理具有挑战性。尽管有关于线粒体疾病患者麻醉管理的报道，但专门针对线粒体心肌病患者心脏麻醉的报道很少。我们报告了一例线粒体心肌病患者，因心功能不全和心脏扩大导致功能性二尖瓣反流，在瑞马唑仑麻醉下成功进行了经皮二尖瓣缘对缘修复术（使用MitraClip™）。
一名57岁女性被诊断为慢性心力衰竭，有10年扩张型心肌病病史。8年前被诊断为线粒体心肌病。在过去2年中她的心力衰竭加重，二尖瓣反流逐渐出现。考虑进行手术干预，但由于她的心功能低下，射血分数为26%，被认为风险太大。因此，选择了经皮植入MitraClip™。在右美托咪定镇静下确保桡动脉和中心静脉置管后，以4mg/kg/h的低剂量瑞马唑仑诱导麻醉。以0.35 - \1.0mg/kg/h的瑞马唑仑和0.1μg/kg/min的瑞芬太尼维持麻醉。术中给予去甲肾上腺素和多巴酚丁胺，手术顺利完成，未发生循环衰竭。患者麻醉后顺利恢复，未出现并发症。术后第8天出院。
对于线粒体心肌病患者植入MitraClip™，瑞马唑仑麻醉管理似乎是安全有效的。
