Apical six chamber view: multiple giant coronary aneurysms associated with IgG4-related disease

Excerpt

A 66-year-old man was brought to our emergency department with sudden lower abdominal pain and hemorrhagic shock. Emergency computed tomography (CT) showed a ruptured internal iliac artery aneurysm with retroperitoneal hemorrhage, and an emergency endovascular aneurysm repair with an iliac branch endo-prosthesis was performed. Postoperative transthoracic echocardiography (TTE) and coronary CT showed diffuse hypo-kinesis of the left ventricle (ejection fraction [EF]: 50%) and mild mitral regurgitation. Notably, a large luminal structure was observed in the interventricular sulcus in an apical four-chamber view (Fig. 1a). Coronary CT revealed a giant coronary artery aneurysm with extensive soft tissue thickening surrounding the coronary arteries (Fig. 1b–d). On laboratory examination, his serum IgG level was 3119 mg/dL (870–1700 mg/dL), IgG4 level was 2360 mg/dL (4.8–105 mg/dL), and antinuclear antibodies was negative. IgG4 subtype made up 76% of the total IgG fraction (normal range: < 5–6%).

…