Ono Kenshi, Nomura Tetsuya, Shoji Keisuke, Kato Yukinori, Wada Naotoshi
Department of Cardiovascular Medicine, Kyoto Chubu Medical Center, 25, Yagi-Ueno, Yagi-cho, Nantan City, Kyoto 629-0197, Japan.
Eur Heart J Case Rep. 2024 Dec 30;9(1):ytae689. doi: 10.1093/ehjcr/ytae689. eCollection 2025 Jan.
Constrictive pericarditis (CP) can arise from various causes, including post-operative degeneration, tuberculosis, and sequelae of pericarditis. Immunoglobulin (Ig) G4-related disease is a rare but recognized cause of CP. However, the specific mechanisms underlying these aetiologies and pathologies remain unclear.
A 67-year-old man presented with a 6-month history of bilateral leg oedema, anorexia, and dyspnoea on exertion. Computed tomography (CT) revealed significant pericardial thickening without calcification, right pleural effusion, and ascites. Echocardiography demonstrated a reduced left ventricular ejection fraction and pericardial thickening. The early diastolic mitral annular tissue velocity (e') was preserved as 11.7 cm/s, despite inferior vena cava dilation. Respiratory variations in mitral inflow velocities and septal bounces were unremarkable. Cardiac catheterization further showed a 'dip and plateau' pattern with equalization of bilateral ventricular end-diastolic pressure. A preliminary diagnosis of CP was made, and pericardiectomy was performed, increasing the cardiac index from 2.0 to 3.0 L/min/m. Pathological examination revealed marked IgG4-positive plasma cell infiltration and tissue fibrosis. Additionally, the patient's post-operative serum IgG4 level was 679 mg/dL. Given these findings, IgG4-related CP without involvement of other organs was determined as the definitive diagnosis. His clinical status improved without requiring corticosteroid therapy.
Optimal therapy for IgG4-related CP remains elusive due to its rarity. Potential therapeutic options include pericardiectomy, pericardiotomy, and corticosteroid therapy. Further examination through the accumulation of similar cases is crucial to establish definitive treatment approaches for this condition.
缩窄性心包炎（CP）可由多种原因引起，包括术后退变、结核以及心包炎后遗症。免疫球蛋白（Ig）G4相关疾病是CP的一种罕见但已被认识的病因。然而，这些病因和病理的具体机制仍不清楚。
一名67岁男性，有6个月双下肢水肿、厌食和劳力性呼吸困难病史。计算机断层扫描（CT）显示心包显著增厚但无钙化，右侧胸腔积液和腹水。超声心动图显示左心室射血分数降低和心包增厚。尽管下腔静脉扩张，但舒张早期二尖瓣环组织速度（e'）保持在11.7 cm/s。二尖瓣血流速度和室间隔跳动的呼吸变化不明显。心导管检查进一步显示“下陷和高原”模式，双侧心室舒张末期压力相等。初步诊断为CP，并进行了心包切除术，心脏指数从2.0提高到3.0 L/min/m。病理检查显示有明显的IgG4阳性浆细胞浸润和组织纤维化。此外，患者术后血清IgG4水平为679 mg/dL。基于这些发现，确诊为不涉及其他器官的IgG4相关CP。他的临床状况改善，无需皮质类固醇治疗。
由于IgG4相关CP罕见，其最佳治疗方法仍不明确。潜在的治疗选择包括心包切除术、心包切开术和皮质类固醇治疗。通过积累类似病例进行进一步研究对于确立这种疾病的明确治疗方法至关重要。
