Department of Internal Medicine, Stony Brook University Hospital, Stony Brook, NY, USA.
Department of Cardiology, Stony Brook University Hospital, Stony Brook, NY, USA.
Am J Case Rep. 2021 Mar 25;22:e929507. doi: 10.12659/AJCR.929507.
BACKGROUND Pheochromocytomas are catecholamine-secreting tumors that develop within the chromaffin cells of the adrenal glands. They most commonly present with hypertension, and the classic triad of symptoms is headaches, palpitations, and diaphoresis. Electrical storm (ES) is defined as at least 3 sustained episodes of ventricular tachycardia (VT), ventricular fibrillation (VF), or appropriate shocks from an implanted cardioverter-defibrillator (ICD) within 24 h. We discuss the case of a 63-year-old man with known bilateral pheochromocytomas who presented with ES prompting multiple ICD shocks, possibly exacerbated by catecholamine surge from his adrenal tumors. CASE REPORT The patient was a 63-year-old man with an extensive medical history including ischemic cardiomyopathy and congestive heart failure with reduced ejection fraction presented with multiple syncopal episodes secondary to persistent monomorphic ventricular tachycardia (MMVT), polymorphic ventricular tachycardia (PMVT), and VF requiring ICD shocks. He had known bilateral pheochromocytomas. ES was attributed to catecholamine excess in the setting of these tumors, so VT ablation was deferred pending tumor removal. Alpha blockade was initiated preoperatively, and the patient subsequently underwent bilateral adrenalectomy; however, he continued to sustain tachyarrhythmias and eventually died despite resuscitative efforts. CONCLUSIONS Bilateral pheochromocytomas are rare adrenal tumors. In even more infrequent situations, they can cause ES secondary to adrenergic stimulation from catecholamine surges. It is worth considering pheochromocytoma in patients with refractory ES, as treating these tumors could potentially reduce the frequency of this dangerous arrhythmia.
嗜铬细胞瘤是一种在肾上腺嗜铬细胞中产生的儿茶酚胺分泌肿瘤。它们最常见的表现是高血压，经典三联征是头痛、心悸和出汗。电风暴（ES）定义为在 24 小时内至少有 3 次持续的室性心动过速（VT）、心室颤动（VF）或植入式心脏复律除颤器（ICD）的适当电击。我们讨论了一名 63 岁男性的病例，该患者患有已知的双侧嗜铬细胞瘤，出现 ES，导致多次 ICD 电击，可能是由肾上腺肿瘤中的儿茶酚胺激增引起的。
患者为 63 岁男性，有广泛的病史，包括缺血性心肌病和充血性心力衰竭伴射血分数降低，因持续性单形性室性心动过速（MMVT）、多形性室性心动过速（PMVT）和 VF 导致多次晕厥而就诊，需要 ICD 电击。他患有已知的双侧嗜铬细胞瘤。ES 归因于这些肿瘤中儿茶酚胺过多，因此在肿瘤切除前推迟了 VT 消融。术前开始进行 α 受体阻滞剂治疗，随后患者接受了双侧肾上腺切除术；然而，他继续出现心动过速，尽管进行了复苏努力，但最终还是死亡。
双侧嗜铬细胞瘤是罕见的肾上腺肿瘤。在更罕见的情况下，它们会因儿茶酚胺激增引起的肾上腺素刺激而导致 ES。对于难治性 ES 的患者，值得考虑嗜铬细胞瘤，因为治疗这些肿瘤可能会降低这种危险心律失常的频率。
