Sato Daisuke, Ochiai Tomoki, Matsumoto Takashi, Mizuno Shingo, Saito Shigeru
Department of Cardiology, Shonan Kamakura General Hospital, 1370-1, Okamoto, Kamakura City, Kanagawa 247-8533, Japan.
Eur Heart J Case Rep. 2024 Aug 13;8(8):ytae425. doi: 10.1093/ehjcr/ytae425. eCollection 2024 Aug.
Systemic sclerosis presents with a variety of cardiac manifestations, while myocarditis is usually a rare finding. Furthermore, there are no reports on the use of mitral transcatheter edge-to-edge repair (M-TEER) for the treatment of severe ventricular functional mitral regurgitation (vFMR) secondary to scleroderma myocarditis.
A-79-year-old male was admitted to our hospital because of fever and fatigue. His physical examination revealed thickening of the fingertips' skin, Raynaud phenomenon, and mild pedal oedema. Positive anti-centromere antibodies indicated a diagnosis of a limited cutaneous systemic sclerosis. He presented with symptoms of heart failure, and moderate to severe lymphocytic infiltration was evident in his endomyocardial biopsy. He responded well to medical therapy and was discharged. However, one month after hospital discharge, he was readmitted to our institution because of worsening heart failure. Transthoracic echocardiography showed a decrease in left ventricular systolic function and progression of left ventricular remodelling, which caused severe vFMR. Endomyocardial biopsy revealed decreased lymphocytic infiltration and mild myocardial interstitial fibrosis, indicative of scleroderma myocarditis. As he was unable to be weaned off inotropes, we performed M-TEER for severe vFMR, which led to a significant reduction in MR volume and improvement of heart failure symptoms. A week after procedure, immunosuppressive therapy was initiated and the patient was discharged home in stable condition.
Scleroderma myocarditis may manifest as heart failure with reduced ejection fraction with severe vFMR. Mitral transcatheter edge-to-edge repair for severe vFMR in the context of myocarditis can be one of the therapeutic options for haemodynamic stabilization.
系统性硬化症有多种心脏表现，而心肌炎通常较为罕见。此外，尚无关于使用二尖瓣经导管缘对缘修复术（M-TEER）治疗硬皮病心肌炎继发的严重心室功能性二尖瓣反流（vFMR）的报道。
一名79岁男性因发热和疲劳入院。体格检查发现指尖皮肤增厚、雷诺现象和轻度足部水肿。抗着丝点抗体阳性提示诊断为局限性皮肤系统性硬化症。他出现心力衰竭症状，心内膜活检显示中度至重度淋巴细胞浸润。他对药物治疗反应良好并出院。然而，出院后一个月，他因心力衰竭加重再次入院。经胸超声心动图显示左心室收缩功能下降和左心室重构进展，导致严重vFMR。心内膜活检显示淋巴细胞浸润减少和轻度心肌间质纤维化，提示硬皮病心肌炎。由于他无法停用正性肌力药物，我们对严重vFMR进行了M-TEER，这导致反流容积显著减少，心力衰竭症状改善。术后一周开始免疫抑制治疗，患者病情稳定后出院。
硬皮病心肌炎可能表现为射血分数降低的心力衰竭伴严重vFMR。在心肌炎背景下，二尖瓣经导管缘对缘修复术治疗严重vFMR可作为血流动力学稳定的治疗选择之一。
