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急性心力衰竭伴偶然发现的肾上腺囊性肿块。

Acute heart failure with incidentally found cystic adrenal mass.

作者信息

Bhat Salman Zahoor, Hamrahian Amir H, Wu Yubo, Han Misop, Salvatori Roberto

机构信息

Division of Endocrinology, Department of Medicine, Diabetes and Metabolism, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.

Division of Urologic Pathology, Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, USA.

出版信息

Endocrinol Diabetes Metab Case Rep. 2023 Oct 24;2023(4). doi: 10.1530/EDM-23-0051. Print 2023 Oct 1.

Abstract

SUMMARY

Pheochromocytomas are rare adrenal tumors characterized by excessive catecholamine secretion. Symptoms and signs associated with pheochromocytomas are usually intermittent and chronic but can rarely develop into life-threatening crises. We describe a case of acute severe congestive heart failure in a previously healthy female, who recovered rapidly (4 days after admission) with acute medical therapy. The etiology on evaluation was a spontaneous bleed in a previously undiagnosed pheochromocytoma, resulting in a pheochromocytoma crisis and transient stress cardiomyopathy, followed by quick recovery of cardiac function. Our aim is to describe pheochromocytoma as a rare cause of stress cardiomyopathy. We discuss the evaluation of pheochromocytoma during critical illness and triggers/treatment strategies for pheochromocytoma crises.

LEARNING POINTS

Hemorrhage in a pheochromocytoma can result in a pheochromocytoma crisis, with sudden release of excess catecholamines resulting in multisystem organ dysfunction and high mortality. Acute decompensated heart failure can be a rare presentation of pheochromocytoma, in a patient with no cardiac risk factors. Measurement of metanephrines in acutely stressful clinical situations can have considerable overlap with the biochemical picture of pheochromocytoma. Early imaging studies may help with the differential diagnosis. Pheochromocytoma should be ruled out before performing an adrenal biopsy. Emergent adrenalectomy in pheochromocytoma crisis results in high mortality. Medical management of the acute crisis followed by elective adrenalectomy after alpha-blockade results in better outcomes.

摘要

摘要

嗜铬细胞瘤是一种罕见的肾上腺肿瘤,其特征为儿茶酚胺分泌过多。与嗜铬细胞瘤相关的症状和体征通常是间歇性和慢性的,但很少会发展为危及生命的危象。我们描述了一例既往健康的女性发生急性严重充血性心力衰竭的病例,该患者通过急性药物治疗迅速康复(入院后4天)。评估发现病因是一个先前未被诊断出的嗜铬细胞瘤自发性出血,导致嗜铬细胞瘤危象和短暂性应激性心肌病,随后心功能迅速恢复。我们的目的是将嗜铬细胞瘤描述为应激性心肌病的一种罕见病因。我们讨论了危重病期间嗜铬细胞瘤的评估以及嗜铬细胞瘤危象的触发因素/治疗策略。

学习要点

嗜铬细胞瘤出血可导致嗜铬细胞瘤危象,过量儿茶酚胺突然释放会导致多系统器官功能障碍和高死亡率。急性失代偿性心力衰竭可能是嗜铬细胞瘤的一种罕见表现,见于无心脏危险因素的患者。在急性应激临床情况下测定甲氧基肾上腺素可能与嗜铬细胞瘤的生化表现有相当大的重叠。早期影像学检查可能有助于鉴别诊断。在进行肾上腺活检前应排除嗜铬细胞瘤。嗜铬细胞瘤危象时紧急肾上腺切除术会导致高死亡率。急性危象的药物治疗,随后在α受体阻滞剂治疗后择期进行肾上腺切除术,可取得更好的治疗效果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2669/10620445/0e63eb702cd4/EDM23-0051fig1.jpg

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