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嗜铬细胞瘤的心血管表现

Cardiovascular Manifestations of Pheochromocytoma.

作者信息

Gu Yu Wei, Poste Jennifer, Kunal Mehta, Schwarcz Monica, Weiss Irene

机构信息

From the Department of Medicine, Division of Endocrinology, New York Medical College/Westchester Medical Center, Valhalla, NY.

出版信息

Cardiol Rev. 2017 Sep/Oct;25(5):215-222. doi: 10.1097/CRD.0000000000000141.

Abstract

Pheochromocytomas are rare endocrine tumors that can have a significant impact on a variety of organ systems, including the cardiovascular system. Although the pathophysiology is not completely understood, pheochromocytomas exert their effects through high levels of catecholamines, mainly epinephrine and norepinephrine, which stimulate adrenergic receptors, including those within the cardiovascular system. Although the most common cardiovascular manifestation is hypertension, patients with pheochromocytoma can present with arrhythmia, hypotension, shock, myocardial ischemia, cardiomyopathy, aortic dissection, and peripheral ischemia. The medical management of the cardiovascular effects of pheochromocytoma is via blockade of adrenergic receptors, usually through the use of alpha blockers, with the addition of beta blockers if needed. However, only surgical resection of the pheochromocytoma is potentially curative, and this tumor requires unique management perioperatively. Because of the variability of presentation and the significant morbidity and mortality of patients with an undiagnosed pheochromocytoma, this entity should not be overlooked in the evaluation of patients with a wide variety of cardiovascular disorders.

摘要

嗜铬细胞瘤是罕见的内分泌肿瘤,可对包括心血管系统在内的多种器官系统产生重大影响。尽管其病理生理学尚未完全明确,但嗜铬细胞瘤通过高水平的儿茶酚胺发挥作用,主要是肾上腺素和去甲肾上腺素,它们刺激肾上腺素能受体,包括心血管系统内的受体。虽然最常见的心血管表现是高血压,但嗜铬细胞瘤患者可出现心律失常、低血压、休克、心肌缺血、心肌病、主动脉夹层和外周缺血。嗜铬细胞瘤心血管效应的药物治疗是通过阻断肾上腺素能受体,通常使用α受体阻滞剂,必要时加用β受体阻滞剂。然而,只有手术切除嗜铬细胞瘤才有可能治愈,并且这种肿瘤在围手术期需要特殊管理。由于临床表现的多样性以及未诊断出的嗜铬细胞瘤患者的高发病率和死亡率,在评估各种心血管疾病患者时不应忽视这一疾病。

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