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.