Mamoojee Yaasir, Arham Munawar, Elsaify Wael, Nag Sath
The James Cook University Hospital, Middlesbrough, UK
The James Cook University Hospital, Middlesbrough, UK.
Clin Med (Lond). 2016 Apr;16(2):201-3. doi: 10.7861/clinmedicine.16-2-201.
Cardiomyopathy as the initial presentation of phaeochromocytoma (PCA) is uncommon. Diagnostic work-up and perioperative management may be challenging within this context. We report three cases of PCA presenting with cardiomyopathy to illustrate the pitfalls in diagnosis and management. None of the patients had typical adrenergic symptoms and all three were established on beta-blockers prior to diagnosis. Their fractionated plasma catecholamine levels were elevated and the diagnosis of PCA was confirmed with various imaging modalities and post adrenalectomy. Interpretation of fractionated catecholamine levels in the context of established cardiomyopathy is difficult as cardiac failure of any aetiology generates an adrenergic response. Hence screening all patients with idiopathic cardiomyopathy is likely to generate a high false-positive rate. However, a high index of suspicion should prompt further diagnostic work-up in patients with idiopathic cardiomyopathy for occult PCAs. Peer-reviewed guidelines are required to guide the investigation and management of suspected catecholamine-induced cardiomyopathy.
以心肌病作为嗜铬细胞瘤（PCA）的首发表现并不常见。在此情况下，诊断检查和围手术期管理可能具有挑战性。我们报告三例以心肌病为表现的PCA病例，以说明诊断和管理中的陷阱。所有患者均无典型的肾上腺素能症状，且三例在诊断前均已服用β受体阻滞剂。他们的血浆儿茶酚胺分级水平升高，通过各种影像学检查及肾上腺切除术后确诊为PCA。在已确诊心肌病的情况下，解读分级儿茶酚胺水平很困难，因为任何病因的心力衰竭都会产生肾上腺素能反应。因此，对所有特发性心肌病患者进行筛查可能会产生较高的假阳性率。然而，高度怀疑应促使对特发性心肌病患者进行进一步诊断检查，以排查隐匿性PCA。需要经过同行评审的指南来指导疑似儿茶酚胺诱导性心肌病的检查和管理。
