Stellate Ganglion Block: A bridge to cervical sympathectomy in refractory Long QT Syndrome

V. Subashini, T. Joseph, S. Nirmal Kumar, S. Khaja Mohideen, K. Senthil Kumar

Department of Anaesthesiology, Kauvery Hospitals, Trichy

Abstract

Long QT syndrome (LQTS) is caused by malfunction of cardiac ion channels impairing ventricular repolarization. Age of presentation can be anywhere from in-utero to adulthood. For patients who continue to develop fatal ventricular arrhythmias, stellate ganglion block prior to cervical sympathectomy is used to predict the efficacy of surgical sympathectomy on Long QT Syndrome management.

Keywords: Long QT Syndrome, Stellate Ganglion, Ventricular Tachycardia

Background

Therapeutic options include beta blockers, calcium channel blockers, implantable cardioverter defibrillators, and left cardiac sympathetic denervation. However there exists no definite guidelines for diagnosis and management. Hence minimally invasive left stellate ganglion block is used to predict the efficacy of surgical sympathectomy on LQTS management

Case Presentation

A 27-year-old postnatal lady (P1L1), delivered a full-term baby in hospital 20 days back. Her postnatal period was complicated by recurrent ventricular tachycardia and ventricular fibrillation (VF) which was reverted by DC shock. As her GCS deteriorated, she had to be intubated. On arrival at our hospital, the patient was drowsy with a pulse rate of 89/min in regular rhythm and BP 100/60mmHg. ECG showed QT interval 570 ms with ventricular premature complex (Fig. 1).

On arrival at our hospital, the patient was drowsy with a pulse rate of 89/min in regular rhythm and BP 100/60mmHg. ECG showed QT interval 570 ms with ventricular premature complex (Fig. 1).

Fig. 1. ECG on arrival QT prolongation.

Her blood investigations were Hb 13.4, Sr Na 145, Sr K 4.55, Sr Mg 2.11, Sr Ca 8.9. She had ejection fraction of 45%, and grade 2 diastolic dysfunction.

She was started on intravenous beta blockers, lignocaine, fentanyl, midazolam and oral mexiletine. In coronary care unit, she developed multiple episodes of transient VF. It was found that the VF was triggered by posterior fascicular ventricular premature complexes. Hence on day 2, the triggering fascicular premature complex was ablated under 2D guidance.

Fig 2. ECG with paced rhythm.

A temporary pacemaker was also placed for overdrive pacing into the coronary sinus by cardiologists. Following the procedure, the patient-maintained sinus rhythm (Fig. 2) and her general condition improved. She was weaned from ventilator and extubated on day 4. On day 6, again she developed ventricular tachycardia which was reverted by DC shock.

Fig. 3. TPI in situ.

Although she was on antiarrhythmic drugs and TPI in situ (Fig 3), she developed multiple transient episodes of ventricular tachycardia. Therefore, she was planned for left stellate ganglion block.

On day 8, ultrasound-guided Left Stellate Ganglion block was performed with ropivacaine 0.75% 8 mL and dexamethasone 0.5 mL. Post-procedure she maintained sinus rhythm with no further episodes of ventricular tachycardia.

As sympathetic denervation showed clinical benefit, surgical cervical sympathectomy was done under general anesthesia.

Post operatively she had no further episodes of ventricular tachycardia. She was discharged home 10 days later. She was asymptomatic on long term follow-up also.

Discussion

Long QT syndrome was initially reported as prolonged QT interval on ECG, in association with deafness (jervell lange nielson syndrome). A few years later similar ECG change without deafness was described (Romano Ward syndrome).

More recently genetic defect from gene encoding ion channels involved in cardiac repolarization are being studied as quoted by Arthur et al [1]..

Symptoms of long QT Syndrome include syncope, loss of consciousness, and sudden death, with ECG showing prolonged QT interval corrected for heart rate (QTc) of more than 0.44 sec. Our case was not associated with deafness, no significant family history, with ECG showing prolonged QTc interval of 570 ms.

The cornerstone of management of LQTS is beta blockers. The antiarrhythmic effect of beta blocker is due to prevention of early after depolarization. Other pharmacological therapies include late sodium channel blockers like Flecainide, Mexiletine. Quinidine is contraindicated since it prolongs QT interval. Digitalis is not an effective treatment.

The patient was started on Betablocker, Lignocaine infusion and oral Mexiletine.

Pharmacological therapy in our patient had no effect on ventricular arrhythmia, hence the next level of therapy – Electrophysiological study with Radio Frequency Ablation- was done. Temporary pacing was inserted but still the patient developed multiple transient episodes of ventricular tachycardia.

A study by Fudim et al [2], observed successful reversal of ventricular arrhythmias following stellate ganglion block.

Stellate (cervicothoracic) ganglion is formed by fusion of inferior cervical ganglion with first thoracic ganglion. The ganglion is contained within the fascial plane of prevertebral fascia, overlying longus colli muscle on either side of cervical vertebra. Sympathetic innervation to the heart comes from both left and right stellate ganglia via post-ganglionic fibers. But left stellate ganglion is quantitatively dominant at the ventricular level, thus explaining the possible efficacy of left stellate ganglion block in terminating VT [3].

In our patient left stellate ganglion block was effective and she had no further episodes of ventricular tachycardia.

Hence the patient was planned for sympathetic denervation. Cervical sympathectomy was done under general anaesthesia, and patient was observed postoperatively and also followed up on long term for any further episodes of arrhythmias.

Conclusion

Long QT syndrome is being recognized with increasing frequency. It requires special attention and careful management. The result of this case report suggests that left stellate ganglion block is a tool to predict the efficacy of surgical sympathectomy on long QT syndrome management

Reference

Arthur AM, et al. Diagnosis, management and therapeutic strategies for long qt syndrome. Heart 2022;108;332-8.
Fudim M, et al. Stellate ganglion block for refractory ventricular arrythmia. JACC Clin Electrophysiol. 2020;6(5):562-71.
Sahoo RK, et al. Stellate ganglion block as rescue therapy in drug resistant electrical strom; Ann Card Anaesth. 2021:23(3);415-8.

Stellate Ganglion Block: A bridge to cervical sympathectomy in refractory Long QT Syndrome