Li Guoliang, Zhao Changying, Wu Lingmin, Yan Yang
Department of Cardiovascular Medicine, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, China.
Department of Cardiovascular Surgery, The First Affiliated Hospital of Xi'an Jiaotong University, No. 277 Yanta West Road, Xi'an, 710061, China.
BMC Cardiovasc Disord. 2025 Mar 7;25(1):157. doi: 10.1186/s12872-025-04601-2.
Arrhythmogenic cardiomyopathy (ACM) is a genetic myocardial disease characterized by progressive myocyte loss and fibrofatty (fibrous and adipose) tissue replacement to predispose these patients to fatal ventricular arrhythmias and impairment of ventricular systolic function. The relationship of ACM and myocarditis has gained significant attention.
This case presented a 28-year-old female who was admitted to the hospital with complaints of recurrent lower limb edema and palpitations for 6 months. Her electrocardiogram revealed a typical manifestation of an advanced form of biventricular arrhythmogenic cardiomyopathy (ACM). Despite systematic medical management, her right ventricle (RV) function deteriorated rapidly, necessitating heart transplantation. Postoperative histopathological examinations confirmed the RV involvement as reflected in the electrocardiogram. Especially, multiple foci of lymphocytic infiltration were observed throughout the heart, with the RV being the most severe.
When a rapid progression of ACM occurs, a concomitant myocarditis should be considered. ACM may be an inflammation-mediated transformation from myocardial tissue to fibrofatty tissue, and myocarditis may be a part of the natural history in some ACM cases.
致心律失常性心肌病（ACM）是一种遗传性心肌疾病，其特征为进行性心肌细胞丢失以及纤维脂肪（纤维性和脂肪性）组织替代，使这些患者易发生致命性室性心律失常和心室收缩功能受损。ACM与心肌炎的关系已受到广泛关注。
本病例为一名28岁女性，因反复下肢水肿和心悸6个月入院。她的心电图显示为晚期双心室致心律失常性心肌病（ACM）的典型表现。尽管进行了系统的药物治疗，但其右心室（RV）功能迅速恶化，需要进行心脏移植。术后组织病理学检查证实了心电图所反映的右心室受累情况。特别是，在整个心脏中观察到多个淋巴细胞浸润灶，其中右心室最为严重。
当ACM迅速进展时，应考虑合并心肌炎。ACM可能是一种由炎症介导的从心肌组织向纤维脂肪组织的转变，在某些ACM病例中，心肌炎可能是其自然病程的一部分。
