辉细胞瘤多系统危象中的心源性休克:病例报告。

Pub Date : 2024-09-09 eCollection Date: 2024-09-01 DOI:10.1093/ehjcr/ytae463
Yun Yun Go, Audrey Jing Ting Ng, Iswaree Devi Balakrishnan, Raj Vikesh Tiwari, Aaron Kian Ti Tong, Lianne Ai Ling Lee, Yann Shan Keh, Donovan Tay
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摘要

背景:辉细胞瘤多系统危象(PMC)的特点是血压不稳定(极度低血压和/或高血压)以及儿茶酚胺过量导致的多器官功能衰竭。初步稳定病情需要药物和/或机械循环支持,然后使用降压药纠正潜在的病理生理学。就诊时,他处于休克状态,多器官功能衰竭。他需要插管、机械通气、双肌力支持和肾脏替代治疗。床旁超声心动图显示,左心室射血分数(LVEF)严重受损,仅为25%。冠状动脉造影显示冠状动脉正常。鉴于炎症指标升高和转氨酶升高,患者接受了腹部/骨盆计算机断层扫描。偶然发现左肾上腺肿块。进一步检查发现,血浆甲肾上腺素和去甲肾上腺素、24 小时尿肾上腺素和去甲肾上腺素均升高。心脏磁共振(CMR)显示心肌炎症和区域室壁运动异常(RWMA)的反向 Takotsubo 模式。诊断结果为继发于 PMC 的心源性休克和应激性心肌病。随后,他开始接受α和β受体阻滞剂以及针对心力衰竭的目标导向药物治疗。68Ga-DOTATATE 扫描显示左侧嗜铬细胞瘤有大量示踪剂摄取。发病 3 周后进行的间期 CMR 显示 LVEF 和 RWMA 接近正常。他成功地接受了腹腔镜左肾上腺切除术,术后无高血压:讨论:临床怀疑PMC是心源性休克的病因需要敏锐的临床判断力,而治疗则需要了解潜在的病理生理学,这需要多学科的参与。
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Cardiogenic shock in phaeochromocytoma multisystem crisis: a case report.

Background: Phaeochromocytoma multisystem crisis (PMC) is characterized by labile blood pressures (extremes of hypo- and/or hypertension) and multiorgan failure as a result of catecholamine excess. Initial stabilization requires pharmacological and/or mechanical circulatory support, followed by the institution of antihypertensives to correct the underlying pathophysiology.

Case summary: A previously well 40-year-old male developed a sudden onset of breathlessness. On presentation, he was in shock with multiorgan failure. He required intubation, mechanical ventilation, dual inotropic support, and renal replacement therapy. Bedside echocardiogram showed a severely impaired left ventricular ejection fraction (LVEF) of 25%. Coronary angiography revealed normal coronary arteries. In view of raised inflammatory markers and transaminitis, a computed tomography abdomen/pelvis was performed. An incidental left adrenal mass was found. Further work-ups revealed raised plasma metanephrine and normetanephrine, 24-h urine epinephrine, and norepinephrine. A cardiac magnetic resonance (CMR) showed myocardial inflammation and reverse Takotsubo pattern of regional wall motion abnormality (RWMA). The diagnosis of cardiogenic shock and stress cardiomyopathy secondary to PMC was made. He was subsequently initiated on α- and β-blockers and goal-directed medical therapy for heart failure. A 68Ga-DOTATATE scan showed avid tracer uptake of the left phaeochromocytoma. An interval CMR 3 weeks from presentation showed near normalization of the LVEF and RWMA. He underwent a successful laparoscopic left adrenalectomy and was antihypertensive-free since.

Discussion: The clinical suspicion for PMC as the cause of cardiogenic shock requires astute clinical judgement, while the management requires an understanding of the underlying pathophysiology that calls for multidisciplinary inputs.

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