Yokoyama Hiroaki, Yamaguchi Masashi, Tobita Kazuki, Saito Shigeru
Department of Cardiology and Catheterization Laboratories, Shonan Kamakura General Hospital, Okamoto 1370-1, Kamakura 247-8533, Japan.
Eur Heart J Case Rep. 2021 Aug 23;5(8):ytab214. doi: 10.1093/ehjcr/ytab214. eCollection 2021 Aug.
Giant cell myocarditis (GCM) is a rare cause of fulminant heart failure (HF). The most common presentation is progressive hemodynamic deterioration, and a few cases present with idiopathic complete atrioventricular block (cAVB). The prognosis of GCM is poor, and GCM patients usually die of HF and ventricular arrhythmia unless cardiac transplantation is performed. Few reports have described the effects of treatments such as immunosuppression and detailed reverse remodelling in GCM patients.
A 69-year-old female presented with cAVB. Transvenous pacemaker was implanted via the left subclavian vein. One and a half months later, she exhibited left ventricular dyssynchrony and lower left ventricular ejection fraction (LVEF), resulting in hospitalization for HF. She received cardiac resynchronization therapy; however, this had no apparently positive effects on her cardiac function. To investigate the cause of the lower LVEF, an endomyocardial biopsy was taken from the right ventricular septum. She was diagnosed with GCM and immediately received immunosuppression therapy with prednisolone and ciclosporin. This resulted in the functional recovery of the right ventricle; on the other hand, the left ventricle had still not recovered based on transthoracic echocardiography. Fortunately, she successfully recovered from severe HF without recurrence.
This is a case of fulminant HF due to GCM which initially presented as cAVB. Moreover, this case demonstrates the quite difference of the functional recovery between the left ventricle and the right ventricle with immunosuppression therapy.
巨细胞性心肌炎(GCM)是暴发性心力衰竭(HF)的罕见病因。最常见的表现是进行性血流动力学恶化,少数病例表现为特发性完全性房室传导阻滞(cAVB)。GCM的预后很差,除非进行心脏移植,GCM患者通常死于HF和室性心律失常。很少有报告描述免疫抑制等治疗方法以及GCM患者详细的逆向重塑效果。
一名69岁女性因cAVB就诊。经左锁骨下静脉植入了经静脉起搏器。一个半月后,她出现左心室不同步和较低的左心室射血分数(LVEF),导致因HF住院。她接受了心脏再同步治疗;然而,这对她的心脏功能没有明显的积极影响。为了探究LVEF降低的原因,从右心室间隔进行了心内膜活检。她被诊断为GCM,并立即接受了泼尼松龙和环孢素的免疫抑制治疗。这导致右心室功能恢复;另一方面,经胸超声心动图显示左心室仍未恢复。幸运的是,她成功从严重HF中康复且未复发。
这是一例因GCM导致的暴发性HF病例,最初表现为cAVB。此外,该病例展示了免疫抑制治疗后左心室和右心室功能恢复的显著差异。