Electrical Storm in a Case of Bilateral Pheochromocytomas

Abstract

Patient: Male, 63-year-old Final Diagnosis: Electrical storm Symptoms: Hypotension • syncope Medication: — Clinical Procedure: — Specialty: Cardiology Objective: Rare disease 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.

Cardiac arrest as initial presentation of pheochromocytoma in a young

“…Owing to its higher affinity for ß1 compared to α1-adrenoceptors, epinephrine has a tendency to cause tachyarrhythmias instead of hypertension, which is in contrast to the action of norepinephrine. 5 Our patient did not experience hypertension despite a significant elevation of norepinephrine in plasma and urine tests. It is possible that undetected transient hypertension crisis may have induced cardiac failure and dilatation.…”

“…[1][2][3] Catecholamines bind with different affinities to adrenoceptors in multiple organs, including the heart and vasculature, leading to tachyarrhythmias and blood pressure changes. [4][5] Although cardiac β1-adrenoceptor stimulation by both norepinephrine and epinephrine causes tachyarrhythmias, the effect on the vasculature is determined by the distribution and affinities. Owing to its higher affinity for ß1 compared to α1-adrenoceptors, epinephrine has a tendency to cause tachyarrhythmias instead of hypertension, which is in contrast to the action of norepinephrine.…”

Emergency Management of Electrical Storm: A Practical Overview

“…Sympathetic nervous system activation is a significant modulatory factor [ 10 ] that contributes to the initiation and maintenance of ES [ 45 ]: sympathetic inhibition plays an important role, especially in subsets of patients with congenital long QT syndrome (LQTS), catecholaminergic polymorphic VT (CPVT) [ 46 , 47 ] and pheochromocytoma [ 48 ]. Some studies supported the role of sympathetic activation, reporting data of higher prevalence of ES on working days and during daytime hours [ 49 ], and even related to stressful events [ 50 ].…”