Kato Mariko, Fujii Takanari, Yamamoto Masayoshi, Kishiro Masahiko, Tomita Hideshi
Pediatric Heart Disease and Adult Congenital Heart Disease Center, Showa University Hospital, Tokyo, Japan.
Department of Radiology, Teikyo University School of Medicine, Tokyo, Japan.
J Cardiol Cases. 2023 Oct 25;29(1):47-49. doi: 10.1016/j.jccase.2023.10.005. eCollection 2024 Jan.
We report the case of a 16-year-old female patient with protein-losing enteropathy that was suspected to be caused by thoracic duct congestion associated with postural compression of right subclavian vein. Non-contrast magnetic resonance lymphangiography showed that the thoracic duct connected to the right-sided venous angle of the right subclavian vein which was obstructed when her right arm was lifted. In this case, comprehensive screening of the lymphatics using non-contrast magnetic resonance lymphangiography, which is a minimally invasive tool with high spatial resolution, was helpful for the recognition of the specific pathophysiology.
Lymphatic disorders associated with congenital heart disease can be fatal. The morphology and dysfunction of the lymphatic system are complicated, and when added to the complex hemodynamics inherent to congenital heart disease, the pathophysiology is more difficult to understand. To understand the complexity of the lymphatic disease, it is necessary to learn a systematic diagnostic process of lymphatic disorders. In the present case, it is beneficial to know the usefulness of non-contrast magnetic resonance lymphangiography to screen overall lymphatics.
我们报告了一例16岁女性蛋白丢失性肠病患者，怀疑是由右锁骨下静脉姿势性压迫相关的胸导管充血引起的。非增强磁共振淋巴造影显示，胸导管连接到右锁骨下静脉的右侧静脉角，当她抬起右臂时该静脉角受阻。在这种情况下，使用非增强磁共振淋巴造影对淋巴管进行全面筛查，这是一种具有高空间分辨率的微创工具，有助于识别特定的病理生理学。
与先天性心脏病相关的淋巴系统疾病可能是致命的。淋巴系统的形态和功能复杂，再加上先天性心脏病固有的复杂血流动力学，其病理生理学更难理解。为了理解淋巴疾病的复杂性，有必要学习淋巴系统疾病的系统诊断过程。在本病例中，了解非增强磁共振淋巴造影对全面筛查淋巴管的有用性是有益的。
