Stellate Ganglion Blockade as Rescue Therapy for Recurrent Torsades de Pointes and as a Bridge to Surgical Sympathectomy in a Pediatric Patient: A Case Report

Abstract

Management of refractory ventricular fibrillation (VF) in patients with implantable implantable cardioverter defibrillator (ICD) presents a therapeutic challenge. We present a case of pediatric refractory ventricular tachycardia (VT)/Torsade de Pointe managed effectively with bilateral stellate ganglion block (SGB) with a long-acting local anesthetic for 18 days as a bridge to more definitive surgical management.

Refractory ventricular arrhythmias are relatively uncommon in pediatric patients, especially those with structurally normal hearts. These arrhythmias are more frequently observed in children with underlying heart conditions, such as cardiomyopathies or inherited ion channel defects.¹ SCN5A channel gene encodes the alpha subunit of Nav1.5. Mutations in SCN5A can alter the structure of the sodium channels, affecting their ability to open and close properly. This disrupts the flow of sodium ions into cardiac cells, which is essential for initiating and conducting electrical impulses. These disruptions can lead to various life-threatening cardiac arrhythmias, such as Brugada syndrome, long QT syndrome (LQTS), and progressive familial heart block.² Management of these arrhythmias often involves a combination of antiarrhythmic medications, catheter ablation, and sometimes implantable cardioverter defibrillator (ICD.)¹

A recent study showed that approximately 50% of patients with refractory ventricular arrhythmia benefit from stellate ganglion block (SGB).^3,4 Originally, 6 case series were published and reported positive results with a total of 103 patients, the largest case series enrolling 30 patients.⁵ STAR study (STellate ganglion block for Arrhythmic stoRm), a multicenter observational study that enrolled 131 patients, received 184 SGB and provided evidence in favor of the effectiveness and safety of SGB for the treatment of refractory electrical storm.⁶ A review article published last year by 2 pioneers in the field calls for a more proactive approach to treat electrical storm by inclusion of SGB in the guidelines and recommendations for treatment of electrical storm.⁵ Literature on pediatric SGB is sparse. There is a mention of 5 case reports involving pediatric patients with refractory ventricular tachycardia (VT) or ventricular fibrillation (VF) associated with LQTS by Savastano and Schwartz,⁵ although details were not provided. Boe et al⁷ also reported a successful treatment of a 7-month-old with histiocytic cardiomyopathy who presented with monomorphic VT, polymorphic VT, and VF for 3 days.

During an electrical storm, while electrical shocks can stabilize a patient’s condition, they may also promote myocardial stunning, heart failure, and proarrhythmic effects. These adverse outcomes are due to mechanisms such as cell damage, metabolic remodeling, Ca²⁺ metabolism anomalies, and inflammatory and profibrotic remodeling. Therefore, early implementation of SGB might prevent myocardial damage induced by both ventricular tachyarrhythmia and defibrillation.⁸

Written Health Insurance Portability and Accountability Act (HIPAA) authorization has been obtained from the patient’s parents.

CASE PRESENTATION

The patient is a 6-year-old male with a history of long QT syndrome type 3 (LQT3) with fetal Torsades de Pointes (TdP), related to a pathologic SCN5A variant and documented TdP at 10 months old, followed by ICD placement in infancy. He had experienced multiple shocks for TdP throughout his lifetime and had undergone pulse generator replacement this previous year.

He presented with upper respiratory tract infection, status epilepticus and was diagnosed with both adenovirus and rhino-enterovirus. He subsequently developed bacterial pneumonia and acute respiratory distress syndrome (ARDS) requiring intubation and eventually venovenous extracorporeal membrane oxygenation (ECMO). After 3 weeks he was decannulated but remained intubated. The course was complicated by ongoing heavy ventricular ectopy burden, nonsustained VT, and TdP requiring multiple ICD shocks, while maximized on multiple antiarrhythmic including lidocaine, mexiletine, nadolol, and esmolol, plus heavy sedated on benzodiazepines, dexmedetomidine, high doses of opioids, and neuromuscular blockade (Figure 1).

Figure 1.

automatic implantable cardioverter defibrillator interrogation result before SGB showing VT episodes and shocks delivered. automatic implantable cardioverter defibrillator indicates xxx; SGB, stellate ganglion block; VT, ventricular tachycardia.

On ICD interrogation, the patient had required 4 shocks from his ICD the day before evaluation for the SGB, and 19 shocks the day before that. We attempted a single shot left SGB. Under ultrasound guidance, a 22-gauge, 4-inch Stimuplex, B. Braun Medical Inc, needle was inserted and 6 mL of 0.25% ropivacaine were administered around the stellate ganglion. There was a significant decrease in ventricular ectopy burden over the next 24 hours (Figure 2). On post-block day 1, the patient experienced only 1 shock delivered by ICD. However, on post-block day 2 the patient again required 9 shocks for refractory TdP.

Figure 2.

Ectopy burden before and after SGB. SGB indicates stellate ganglion block.

We subsequently placed SGB with continuous infusions of ropivacaine at 1 mL/h bilaterally and subsequently titrated to 1.5 mL/h (Figure 3). He received no further shocks for the next 18 days. Based on this response, the decision was made to pursue surgical cardiac sympathetic denervation (CSD) as the stellate catheters could not be maintained for an extended period. The right-sided catheter was removed on post-block day 10 due to concerns about catheter site contamination. On day 18, the left-sided catheter was removed before operation for surgical CSD (excision of left sympathetic chain T1–T5 was performed but the right sympathetic chain could not be visualized due to multiple adhesions related to the patient’s prior ARDS). In the immediate postoperative period, the patient remained hemodynamically stable, without further ICD shocks although a functional 2:1 AV block was repeatedly observed over the first 48 hours that was refractory to ventricular pacing. Unfortunately, by postsympathectomy day 8, the patient received multiple ICD shocks daily and underwent evaluation for heart transplant. The case reported in this paper adhered to case report guidelines.

Figure 3.

Bilateral stellate ganglion catheter. stellate ganglion indicates xxx.

DISCUSSION

Indications for SGB include chronic pain (oral/neck pain), complex regional pain syndrome, posttraumatic stress disorder, and more recently for refractory cardiac arrhythmia. This case highlights that SGB may provide treatment for patients who are experiencing TdP related to LQTS. American heart association and American college of cardiology (AHA/ACC) guideline recommendations give a class IIB indication to autonomic neuromodulation in patients with ventricular arrhythmia refractory to pharmacological management and in whom catheter ablation is not feasible.⁹

The paratracheal approach with or without fluoroscopy is the dominant approach to SBG. Ultrasound-guided approach to SGB has been used to improve visualization of the needle and spread of local anesthetic. This method increases the accuracy of the block and minimizes potential needle damage to structures in vicinity of the stellate ganglion. Proximity of stellate ganglion to vascular structures, brachial plexus, Vagus nerve, and its branches, as well as esophagus warrants expertise and training in performing the procedure. Performing pediatric SGB is technically more challenging compared to adults, due to the smaller size of a child’s neck and the proximity of vital structures. We believe using ultrasound guidance in pediatric SGB is superior to blind paratracheal and fluoroscopy-guided approach to improve accuracy and safety, while also avoiding the negative effects of X-ray exposure.

If placement of the catheter for continuous infusion is the goal, percutaneous ultrasound approach is by far the preferred technique. We named our approach “Transscalene approach to stellate ganglion” and will report a detailed description of this technique (which has been used in >50 cases for different indications) in a future article. By first locating anatomical landmarks and identifying all important structures in different sono-anatomical views, we advance the needle via in-plane approach through the skin and subcutaneous tissue into the middle scalene muscle and pop through the medial border of scalene muscle making sure the needle tip reaches behind the carotid artery and then inject a local anesthetic at approximately the C7 nerve root level. We also placed a catheter through the needle and confirmed the position of the catheter tip by ultrasound. Bupivacaine is widely used and well-studied, making it the first choice for many procedures. There is no information about the differential blockade of bupivacaine, ropivacaine, or levobupivacaine on sympathetic nerves and ganglia versus sensory or motor neurons. Latter choices should be selected to decrease the chances of local anesthetic toxicity.

In conclusion, this case shows that SGB could eliminate refractory VT/TdP in the case of LQT3 and significantly reduce ventricular ectopy burden when maximal medical management failed to suppress sympathetic drive and arrhythmia. We learned that continuous high concentrations of local anesthetic infusions close to the stellate ganglion were required to eliminate TdP and single injection was ineffective after 24 hours. Notable in this case is the duration that the catheters were kept in place without any immediate or long-term complication, which allowed us to optimize the patient for surgical CSD. Based on the favorable response of the patient to SGB, the decision could be made to seek a surgical approach more confidently. However in this case, the arrhythmia did not resolve with left CSD.

Finally, SGB could be a potential treatment for patients with refractory TdP due to underlying cardiac conditions, including in pediatric patients. Further research is needed to evaluate the safety and efficacy of SGB via either catheter or single-shot block in patients with refractory torsade in a larger group of patients.

ACKNOWLEDGMENTS

We express our deep gratitude to Professor Kalyanam Shivkumar for his invaluable encouragement, guidance, and support throughout the entire process of preparing this article.

This manuscript was handled by: Christina L. Jeng, MD, FASA.