Fennell David, Miller Clare, Ludgate Stephen, Conneely John, O'Brien Serena, Conrick-Martin Ian, Hastings Jennifer, McQuaid Siobhán E
Department of Endocrinology, Mater Misericordiae University Hospital, Dublin, Ireland.
Department of Surgery, Mater Misericordiae University Hospital, Dublin, Ireland.
Endocrinol Diabetes Metab Case Rep. 2023 Apr 19;2023(2). doi: 10.1530/EDM-22-0392. Print 2023 Apr 1.
Phaeochromocytoma, a rare neuroendocrine tumour of chromaffin cell origin, is characterised by catecholamine excess. Clinical presentation ranges from asymptomatic disease to life-threatening multiorgan dysfunction. Catecholamine-induced cardiomyopathy is a dreaded complication with high lethality. While there is lack of evidence-based guidelines for use of veno-arterial extracorporeal membrane oxygenation (V-A ECMO) in the management of this condition, limited to case reports and small case series, V-A ECMO has been reported as 'bridge to recovery' therapy, providing circulatory support in the initial period of stabilisation prior to surgery. We report on two patients presenting with catecholamine-induced cardiomyopathy and circulatory collapse who were successfully treated with V-A ECMO for 5 and 6 days, respectively, providing initial haemodynamic support. After stabilisation and introduction of alpha-blockade, both cases had favourable outcomes, with successful laparoscopic adrenalectomies on days 62 and 83 of admission, respectively. Our case reports provide further support for the use of V-A ECMO in the treatment of such gravely ill patients.
Phaeochromocytoma should be considered in the diagnosis of patients presenting with acute cardiomyopathy. Management of catecholamine-induced cardiomyopathy is complex and requires multidisciplinary specialist input. Pre-operative management of phaeochromocytoma involves alpha-blockade; however, haemodynamic instability in the setting of cardiogenic shock can preclude alpha-blockade use. Veno-arterial extracorporeal membrane oxygenation is a life-saving intervention which may be considered in cases of acute catecholamine-induced cardiomyopathy and cardiogenic shock in order to provide the required haemodynamic support in the initial phase of treatment, enabling the administration of traditional pharmacological agents, including alpha-blockade.
嗜铬细胞瘤是一种罕见的起源于嗜铬细胞的神经内分泌肿瘤，其特征是儿茶酚胺过量。临床表现从无症状疾病到危及生命的多器官功能障碍不等。儿茶酚胺诱导的心肌病是一种可怕的并发症，致死率很高。虽然在这种疾病的管理中缺乏关于使用静脉-动脉体外膜肺氧合（V-A ECMO）的循证指南，相关报道仅限于病例报告和小型病例系列，但V-A ECMO已被报道为“恢复之桥”疗法，在手术前的初始稳定期提供循环支持。我们报告了两名患有儿茶酚胺诱导的心肌病和循环衰竭的患者，他们分别接受V-A ECMO成功治疗5天和6天，获得了初始血流动力学支持。在病情稳定并引入α-阻滞剂后，两例患者均取得了良好的预后，分别在入院第62天和第83天成功进行了腹腔镜肾上腺切除术。我们的病例报告为V-A ECMO用于治疗此类重症患者提供了进一步的支持。
在诊断急性心肌病患者时应考虑嗜铬细胞瘤。儿茶酚胺诱导的心肌病的管理很复杂，需要多学科专家的参与。嗜铬细胞瘤的术前管理包括使用α-阻滞剂；然而，心源性休克情况下的血流动力学不稳定可能会妨碍使用α-阻滞剂。静脉-动脉体外膜肺氧合是一种挽救生命的干预措施，对于急性儿茶酚胺诱导的心肌病和心源性休克病例，可以考虑使用，以便在治疗的初始阶段提供所需的血流动力学支持，从而能够使用包括α-阻滞剂在内的传统药物。
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