Zhou Fang-Fang, Ding Jia-Sheng, Zhang Min, Tian Xin
Department of Ultrasound, Lishui Central Hospital, The Fifth Affiliated Hospital of Wenzhou Medical University, Lishui 323000, Zhejiang Province, China.
Department of Intensive Care Unit, Lishui Central Hospital, The Fifth Affiliated Hospital of Wenzhou Medical University, Lishui 323000, Zhejiang Province, China.
Open Med (Wars). 2022 Aug 10;17(1):1368-1375. doi: 10.1515/med-2022-0535. eCollection 2022.
Paragangliomas are rare neuroendocrine tumors that originate in the chromaffin cells of the adrenal medulla or lymph nodes. Paragangliomas manifest in rare cases as catecholamine crisis, leading to heart failure, intracranial hemorrhage, renal failure, arrhythmias, pulmonary edema, or multisystem failure. Takotsubo cardiomyopathy is also called apical ballooning syndrome or stress cardiomyopathy. Left ventricular dysfunction with apical hyperkinesis and basilar and midventricular akinesis in the absence of coronary artery disease is highly suggestive of a variant of stress cardiomyopathy (inverted takotsubo cardiomyopathy). Herein, we report the case of a 69-year-old man with an unknown retroperitoneal paraganglioma who suffered from cardiogenic shock due to inverted takotsubo cardiomyopathy. He was treated with venoarterial extracorporeal membrane pulmonary oxygenation (ECMO) in combination with an intra-aortic balloon pump. After the restoration of cardiac function, a successful transition to curative retroperitoneal paraganglioma resection was performed. We conclude that ECMO is a valuable option for undiagnosed endocrine emergencies, helping to restore cardiac function and allowing sufficient time for further accurate diagnosis and specific treatment.
副神经节瘤是一种罕见的神经内分泌肿瘤，起源于肾上腺髓质或淋巴结的嗜铬细胞。副神经节瘤在罕见情况下表现为儿茶酚胺危象，导致心力衰竭、颅内出血、肾衰竭、心律失常、肺水肿或多系统衰竭。Takotsubo心肌病也称为心尖球囊综合征或应激性心肌病。在无冠状动脉疾病的情况下，出现心尖运动亢进以及基底和心室中部运动减弱的左心室功能障碍高度提示应激性心肌病的一种变异型（倒置型Takotsubo心肌病）。在此，我们报告一例69岁男性患者，其患有不明的腹膜后副神经节瘤，因倒置型Takotsubo心肌病导致心源性休克。他接受了静脉-动脉体外膜肺氧合（ECMO）联合主动脉内球囊泵治疗。心脏功能恢复后，成功过渡到根治性腹膜后副神经节瘤切除术。我们得出结论，ECMO对于未确诊的内分泌急症是一种有价值的选择，有助于恢复心脏功能，并为进一步准确诊断和特异性治疗留出足够时间。
