Discussion:
Brugada Syndrome is an inherited cardiac electrical disorder occurring in the absence of obvious structural heart disease, leading to right bundle branch block, ST elevation and sudden cardiac death due to polymorphic ventricular tachycardia. Patients with Brugada Syndrome can suffer from electrical storms [5]. The stellate ganglion is a collection or group of sympathetic nerve fibers responsible for innervating the arms, face, and chest. During an ES, the excess catecholamine release from sympathetic fibers can lead to sympathetic hyperactivity outflow to the heart resulting in potentially fatal arrhythmias [4] [3].
Studies have shown benefit in restoring systemic circulation using ECMO in patients with cardiogenic shock related to ES and hemodynamic instability in ventricular arrhythmias [3]. ECMO support can stabilize the patient in order to facilitate future surgical interventions in more favorable hemodynamic conditions. Despite documented benefits, there appears to be an increase in mortality that is directly proportional to length of time on ECMO [6]. This supports the use of ECMO as a bridge to intervention rather than for prolonged survival in patients with cardiogenic shock.
Stellate Ganglion Block is traditionally performed by injecting local anesthetics percutaneously to the stellate ganglion. Though SGB has been shown to be effective, its therapeutic effectiveness and duration is variable depending on the type of anesthesia and method of administration [7]. This variability is related to the pharmacodynamics of the agents used, site of injection, and thickness of the ganglion sheath.
CSD describes the surgical resection of the majority or the lower half of the stellate ganglion as well as the sympathetic chains from T1-T4. CSD interrupts the major source of norepinephrine released to the heart and has multiple antiarrhythmic effects including increasing the threshold for ventricular fibrillation and increasing ventricular refractory period [8]. When standard medical treatments fail, Wilde et al. demonstrated the effectiveness of left sided CSD in patients who suffered from catecholaminergic polymorphic VT [8]. Symptoms were controlled for up to 20 years postoperatively in some patients, although ventricular arrhythmias did occur at high workloads. Left Cardiac Sympathetic Denervation (LCSD) is more commonly performed, but Bilateral Cardiac Sympathetic Denervation (BCSD) or right sided CSD (RCSD) performed as an adjunct to a previously failed LCSD has also been performed with promising results [9] [10]. In canine studies comparing left, right, or bilateral sympathectomy, the most profound anti-arrhythmic effects were seen with bilateral sympathectomy [11]. Despite being more invasive, bilateral CSD appears to provide a more definitive and effective therapeutic intervention. It can prevent catecholamine surges and decrease the likelihood of potentially life-threatening arrhythmias that are refractory to defibrillation.
Conclusion :
We present this case in order to review a rare condition and suggest that in cases when routine management of ES, such as defibrillation, anti-arrhythmic therapies, and ablation, fail to control life-threatening ventricular arrhythmias, ECMO support followed by CSD can be an effective and definitive treatment option. All of these patients should undergo surgical intervention with an experienced cardiothoracic surgeon.