Bak Minjung, Lee Jinyoung, Hong Minseok, Kim Darae, Choi Jin-Oh, Kim Jung-Sun, Yang Jeong Hoon
Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
J Korean Med Sci. 2025 Jul 28;40(29):e221. doi: 10.3346/jkms.2025.40.e221.
Giant cell myocarditis (GCM) is a rare but severe disease with high morbidity and mortality, often requiring heart transplantation (HT). While immunosuppressive therapy has improved outcomes, recurrence after HT remains high, and long-term survival without transplantation is also limited. This case series presents five biopsy-confirmed GCM cases, highlighting clinical characteristics and strategies to improve prognosis. A retrospective analysis was conducted on five GCM patients at Samsung Medical Center (2004-2024). The median age was 57.8 years, and 40% had underlying autoimmune diseases. All patients presented with fulminant heart failure, requiring vasopressor support or mechanical circulatory support (MCS). Early diagnosis facilitated targeted therapy, improving outcomes in select cases. Two patients achieved long-term survival without recurrence. One patient recovered cardiac function and avoided in-hospital mortality but was later readmitted due to infection and subsequently died. The remaining two patients underwent HT but died during hospitalization due to post HT complications. Timely histologic confirmation, early immunosuppressive therapy, and appropriate MCS bridging are critical in GCM management. Larger studies are needed to validate these findings.
巨细胞性心肌炎（GCM）是一种罕见但严重的疾病，发病率和死亡率都很高，通常需要进行心脏移植（HT）。虽然免疫抑制治疗改善了治疗效果，但心脏移植后复发率仍然很高，未经移植的长期生存率也很有限。本病例系列介绍了5例经活检确诊的GCM病例，突出了其临床特征以及改善预后的策略。对三星医疗中心的5例GCM患者（2004年至2024年）进行了回顾性分析。中位年龄为57.8岁，40%的患者有潜在的自身免疫性疾病。所有患者均表现为暴发性心力衰竭，需要血管升压药支持或机械循环支持（MCS）。早期诊断有助于进行靶向治疗，在部分病例中改善了治疗效果。两名患者实现了无复发的长期生存。一名患者恢复了心脏功能，避免了院内死亡，但后来因感染再次入院，随后死亡。其余两名患者接受了心脏移植，但在住院期间因心脏移植后并发症死亡。及时的组织学确诊、早期免疫抑制治疗以及适当的MCS桥接在GCM的管理中至关重要。需要开展更大规模的研究来验证这些发现。
