Zhou Xiang, Liu Dawei, Su Longxiang, Long Yun, Du Wei, Miao Qi, Li Fang, Jin Zhengyu, Zeng Zhengpei, Luo Ailun, Huang Yuguang
From the Department of Critical Care Medicine (XZ, DL, LS, YL, WD); Department of Cardiac Surgery (QM); Department of Nuclear Medicine (FL); Department of Radiology (ZJ); Department of Endocrinology (ZZ); and Department of Anesthesiology (AL, YH), Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China.
Medicine (Baltimore). 2015 May;94(17):e790. doi: 10.1097/MD.0000000000000790.
Cardiac pheochromocytoma is relatively rare. Few reports describe the intraoperative and postoperative progression of patients experiencing a life-threatening pheochromocytoma crisis treated with extracorporeal membrane oxygenation (ECMO).A 35-year-old man was referred to our facility for paroxysmal hypertension with a 10-year history of sweating, headaches, cardiac palpitations, and postexercise dyspnea. The patient initially underwent urine catecholamine measurement and an isotope scan, somatostatin receptor scintigraphy, and 18F-fluorodeoxyglucose positron emission tomography/computer tomography (CT), which indicated a multiple, cardiac pheochromocytoma. Echocardiography, cardiac magnetic resonance imaging (MRI), CT reconstruction, and a coronary CT angiography revealed several lesions at the aortic root and along the cardiac vasculature.Multifocal cardiac pheochromocytoma was diagnosed and pheochromocytoma crisis with severe cyclic blood pressure fluctuation occurred during surgery.Surgical resection of multiple pheochromocytomas in the right medial carotid sheath, mediastinum between the main and pulmonary arteries, and between the abdominal aorta and inferior vena artery was performed. To ensure cardiac perfusion and avoid severe circulatory fluctuation, the cardiac paraganglioma resection was prioritized. After resecting the cardiac pheochromocytoma, a severe pheochromocytoma crisis with rapid cyclic blood pressure fluctuation developed. ECMO and intraaortic balloon pump (IABP) were initiated to stabilize circulation and perfusion. Phenoxybenzamine, norepinephrine, epinephrine, and fluid resuscitation were administered to support cardiovascular function.The magnitude of blood pressure fluctuation steadily decreased with treatment. IABP was discontinued after 3 days, and ECMO was discontinued after 16 days. The patient was discharged 3 months postoperatively.This case indicates that mechanical life support with ECMO is a valuable option for pheochromocytoma-induced cardiac shock and should be considered as an effective therapeutic choice in patients with highly unstable hemodynamic function.
心脏嗜铬细胞瘤相对罕见。很少有报告描述接受体外膜肺氧合（ECMO）治疗的危及生命的嗜铬细胞瘤危象患者的术中及术后病情进展。一名35岁男性因阵发性高血压被转诊至我院，有10年出汗、头痛、心悸及运动后呼吸困难病史。患者最初接受了尿儿茶酚胺测定、同位素扫描、生长抑素受体闪烁显像及18F-氟脱氧葡萄糖正电子发射断层扫描/计算机断层扫描（CT），结果提示为多发性心脏嗜铬细胞瘤。超声心动图、心脏磁共振成像（MRI）、CT重建及冠状动脉CT血管造影显示主动脉根部及沿心脏血管系统有多个病灶。诊断为多灶性心脏嗜铬细胞瘤，术中发生了伴有严重周期性血压波动的嗜铬细胞瘤危象。对右侧颈总动脉鞘内侧、主肺动脉之间的纵隔以及腹主动脉与下腔静脉之间的多个嗜铬细胞瘤进行了手术切除。为确保心脏灌注并避免严重的循环波动，优先进行了心脏副神经节瘤切除术。切除心脏嗜铬细胞瘤后，出现了伴有快速周期性血压波动的严重嗜铬细胞瘤危象。启动了ECMO和主动脉内球囊泵（IABP）以稳定循环和灌注。给予酚苄明、去甲肾上腺素、肾上腺素及液体复苏以支持心血管功能。随着治疗，血压波动幅度稳步下降。IABP在3天后停用，ECMO在16天后停用。患者术后3个月出院。该病例表明，ECMO机械生命支持对于嗜铬细胞瘤所致的心源性休克是一种有价值的选择，对于血流动力学功能高度不稳定的患者应被视为一种有效的治疗选择。
