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Original Articles: Pediatric Cardiac

Video-Assisted Thoracoscopic Left Cardiac Sympathetic Denervation: A Reliable Minimally Invasive Approach for Congenital Long-QT Syndrome

^a Department of Thoracic Surgery, Peking University, People's Hospital, Beijing, China
^b Department of Cardiology, Peking University, People's Hospital, Beijing, China

Accepted for publication July 29, 2008.

^_* Address correspondence to Dr Wang, Department of Thoracic Surgery, People's Hospital of Peking University, Beijing, 100044, China (Email: jwangmd{at}yahoo.com).

	Abstract

Top Abstract Introduction Patients and Methods Results Comment References

 
Background: The purpose of this study was to assess the feasibility and long-term effect of video-assisted thoracoscopic left cardiac sympathetic denervation for congenital long-QT syndrome.

Methods: From December 2002 to May 2007, 11 patients who could not tolerate or who were refractory to β-blocker therapy received video-assisted thoracoscopic left cardiac sympathetic denervation. Under general anesthesia, the pleural cavity was entered through three 1.5-cm incisions in the left subaxillary area. The left thoracic sympathetic chain was identified, and the lower one third of the left stellate ganglion, together with T₂ to T₅ sympathetic chain, was resected.

Results: The mean operative time was 40.9 ± 7.7 minutes. Blood loss was minimal. The mean postoperative stay was 6 ± 1.4 days. There were no major perioperative complications apart from mild ptosis of the left upper eyelid in 1 patient who subsequently recovered shortly after the procedure. The mean follow-up time was 37.0 ± 26.3 months. Seven of the patients are totally free of cardiac events and report good quality of life. One patient experienced decreased syncopal events from 5 or 6 times per year to 2 or 3 times per year. One patient still experiences syncopal events 3 to 4 times a year, but with shortened duration to several seconds. One patient reports syncope 10 times per year. Only 1 patient died, early in the second year after surgery. In conclusion, the overall efficacy rate (that is, reduction in syncopal episodes) is 81.8% (9 of 11) and the mortality rate, 9.1% (1 of 11).

Conclusions: Video-assisted thoracoscopic left cardiac sympathetic denervation is a simple and minimally invasive technique that results in good long-term benefits in patients with congenital long-QT syndromes.

	Introduction

Top Abstract Introduction Patients and Methods Results Comment References

 
Congenital long-QT syndrome is a rare but severe cardiac disease that carries a risk of life-threatening ventricular arrhythmias or sudden cardiac death [1]. Clinical observations and studies in animals have demonstrated that a sudden increase in sympathetic activity is the main cause of onset of these life-threatening arrhythmias. Current therapeutic options include β-blockers, implantable cardioverter-defibrillators (ICDs), and permanent pacing [1, 2]. Beta-blockers have been considered as the initial and primary therapy, but not all patients have been demonstrated to benefit from this intervention. Nearly 1 of 4 have no effect or cannot tolerate β-blocker therapy [1, 3]. Left cardiac sympathetic denervation (LCSD) by resection of T1 to T5 ganglions is an alternative therapy for patients who are resistant to or who have failed β-blocker therapy [1, 4, 5].

Implantable cardioverter-defibrillator therapy is an alternative for high-risk patients. However, ICDs may not be practical because of the high cost and the relatively short lifespan of the devices. In patients with frequent ICD shocks or in those at high risk for sudden cardiac death in whom ICD placement cannot be performed, LCSD may be indicated [2]. Left cardiac sympathetic denervation has been shown to be a safe and effective procedure to decrease lethal arrhythmias and cardiac death, but its clinical use has been hindered by the complexity of the operative complications. Its current use is limited to a small number of medical centers, mainly in Europe, and the total number of cases reported is less than 200 [2].

Video-assisted thoracoscopic (VATS) LCSD was first reported by Reardon and colleagues [6] in 2000 and by us in 2003 [7]. Since 2002, we have performed 11 such procedures and here report our long-term results. The feasibility and effectiveness of this new approach will be reviewed in this paper.

	Patients and Methods

Top Abstract Introduction Patients and Methods Results Comment References

 
Table 1 shows patient data.

Surgical Techniques
The patient was placed in a right lateral decubitus position. General anesthesia was used in all patients. Surface defibrillator electrodes were routinely placed. The pleural cavity was entered through three 1.5-cm incisions at the fourth intercostal space on the anterior axillary line and the third and fifth intercostal space on the midaxillary line. A 10-mm, 30-degree thoracoscope (Stryker Endoscopy, Santa Clara, California) was inserted through the fourth intercostal space incision while surgical instruments were placed through the other two incision sites.

The sympathetic chain running vertically over the necks of the ribs in the costovertebral region was easily identified under the transluminal parietal pleura. The locations of sympathetic ganglia of the first six segments were identified corresponding to the number of ribs. The left stellate ganglion was located in the first intercostal space and was much larger than the others, as it usually consisted of C7, C8, and T1 ganglia. Thus, the range of resection included the left T2 through T5 sympathetic chain and the lower third of the stellate ganglion.

The inferior pole of the T5 ganglion was first isolated and transected. The sympathetic chain was then isolated upward with all the branches either anterior or posterior sequentially cut off. The left stellate ganglion was relatively easy to identify with this sequence, and the lower third of the ganglion was clipped and then transected. Branches from the lower part of stellate ganglion above the second rib were preserved to avoid Horner's syndrome. Electrocautery must also be avoided near stellate ganglion to prevent injury and Horner's syndrome. Chest tubes were no longer needed after complete reexpansion of the left lung by the end of the operation. A chest radiograph was obtained on the first postoperative day to ensure complete lung expansion. Oral β-blocker therapy was maintained at the same dosage before surgery for at least 12 months. Follow-up was scheduled every 3 months. Drug dosage was slightly decreased in the second year if symptoms permitted, but were maintained at acceptable levels throughout the study.

Statistics
Twelve-lead and 24-hour Holter monitoring electrocardiograms were recorded 24 hours before and after LCSD. Treadmill exercise tests were also performed before and 6 days after surgery to assess changes in heart rate and the QT interval. The paired t test was used for statistical analysis.

	Results

Top Abstract Introduction Patients and Methods Results Comment References

 
The mean operative time was 40.9 ± 7.7 minutes (range, 35 to 55), and the blood loss was minimal. All patients were able to move freely several hours after surgery. None of them experienced arrhythmias before discharge. The mean postoperative stay was 6 ± 1.4 days (essentially delayed owing to postoperative electrophysiologic studies). One patient experienced mild postoperative left eyelid ptosis, which recovered in 6 days. No patients experienced Horner's syndrome, although all subjects reported decreased sweating on the left side of face and trunk, which did not influence their quality of life.

The mean follow-up time was 37.0 ± 26.3 months. Seven of the subjects are totally free of cardiac events and experience good quality of life. One subject experienced decreased syncopal events from 5 or 6 times per year to 2 or 3 times per year. One subject experienced syncopal events 3 to 4 times a year, but with shortened duration of several seconds. One experienced syncopal events as often as 10 times per year. One patient (a 6-year-old boy) who used to have frequent syncope as often as three times a day before surgery died during the second postoperative year while playing, even though he was eventless in the first year postoperatively. This patient was only able to tolerate up to 10 mg propranolol per day, and his electrocardiogram had documented ventricular arrhythmia every day before surgery. Even during the surgery, ventricular tachycardia occurred when the sympathetic chain was being isolated. The clip of the stallete ganglion immediately stopped the ventricular tachycardia. The patient was discharged 7 days later without any malignant arrhythmias.

Overall, the frequency of syncopal events in the subjects was 7.6 ± 8.6 before surgery, and was 1.6 ± 3.1 times per year after surgery, reduced by 73.8%, showing a nearly significant difference (t = 1.971; p = 0.080). The overall efficacy rate was 81.8%, and the mortality rate was 9.1%.

	Comment

Top Abstract Introduction Patients and Methods Results Comment References

 
Surgical Treatment for Long-QT Syndrome
Lethal ventricular arrhythmias in long-QT patients can be triggered by sudden increases in sympathetic activity [4, 14]. One of the characteristics of long-QT syndrome, T-wave alternant, can be reproduced by stimulation of the left stellate ganglion in animals and in humans [8–10]. Left stellectomy in animals markedly increases ventricular fibrillation threshold and prolongs ventricular refractoriness [11]. The use of LCSD was first reported by Moss and McDonald [12] in 1971 to treat patients with long-QT syndrome who were refractory to pharmacologic therapy. Accordingly, the initial procedure was known as left stellectomy. Although it provided only limited cardiac denervation in humans and was often associated with Horner's syndrome, this procedure built the foundation of surgical treatment for this syndrome [5]. In 1976, Schwartz and colleagues [11] and Moss and McDonald [12] introduced a new procedure known as high thoracic left sympathectomy. With high thoracic left sympathectomy, the lower part of the left stellate ganglion together with the first four or five left thoracic ganglia are removed. This procedure produces an adequate cardiac sympathetic denervation and is associated with a very low incidence of Horner's syndrome.

For these reasons, high thoracic left sympathectomy has become a standard option for LCSD. Schwartz and coworkers [2] recently assessed LCSD in 147 long-QT syndrome patients at especially high risk of death; 99% of them were symptomatic and 48% had had cardiac arrest, and 75% of those treated with β-blockers continued to have cardiac events. The mean follow-up after surgery was 8 years. After LCSD, 46% of patients became asymptomatic, syncope occurred in 31%, cardiac arrest was aborted in 16% and sudden death in 7%. The mean yearly number of cardiac events decreased by 91% (p < 0.001).

Anatomy of Stellate Ganglion
The recent study of Liu and colleagues [13] found that, among Chinese people, the average size of the stellate ganglion was 20.8 ± 3.1 mm by 8.5 ± 1.9 mm. The fusion rate was 60.0% (36 of 60). In 33.3% of the population, the lower part of the stellate ganglion was lower than the upper border of the second rib. Rami from the T1 spine nerve entered the stellate ganglion above the upper border of the second rib in all cases. Therefore, transection of the sympathetic trunk at the level of the upper border of the second rib can only damage the lower portion of the stellate ganglion and will not induce any optic symptoms such as in Horner's syndrome.

Approach Selection
The dominant approach for left cardiac sympathectomy is through an anterior superclavicular incision. All or part of the left stellate ganglion and the most upper left thoracic sympathetic ganglia are resected without entry into the pleural cavity. This technique is rather sophisticated and is associated with Horner's syndrome in as many as 100% of patients [1, 12]. Another approach is posterior thoracotomy [14]. These two approaches can cause pronounced pain and may leave significant incision scars. A new approach referred to as left thoracoscopic sympathectomy and stellate ganglionectomy was introduced in 2000 [6]. Based on these considerations, we advocate VATS-LCSD.

Video-Assisted Thoracoscopic LCSD
Video-assisted thoracoscopy has been used for upper thoracic sympathectomy for palmar hyperhidrosis since the early 1990s [15]. The minimally invasive sympathectomy techniques including incisions arrangement and sequence of dissection have undergone progressive refinement in recent years. In our center, the procedure of VATS-LCSD is now completed in less than 40 minutes with minimal complications. The VATS-LCSD technique is one of the simplest procedures, and is readily performed by surgeons who have received basic training in video-assisted thoracoscopy. The long-term follow-up results of our data have shown an efficacy rate as high as 81.8% (9 of 11).

Indications for VATS-LCSD
Left cardiac sympathetic denervation is associated with a significant reduction in the incidence of aborted cardiac arrest and syncope in high-risk long-QT syndrome patients. Although QT intervals may not change, the slope between QT and RR, both in the before and aftre exercise slopes as well as in the interval, becomes less steep after LCSD. These findings suggest that the exaggerated delay in repolarization with decreasing heart rate for long-QT syndrome patients is improved by LCSD. This may be one of the mechanisms of LCSD efficacy for treating long-QT syndrome patients [16]. This procedure is thought to be effective in some genotypes with high sympathetic activities such as LQT1 and LQT3.

However, LCSD is not entirely effective in preventing cardiac events, including sudden death, during long-term follow-up [2]. An ICD is thought to be life-saving for patients with previous cardiac arrest, but it also has limitations. The ICDs do not prevent arrhythmic episodes, so they can not absolutely prevent sudden death. Furthermore, frequent ICD shocks may cause severe mental distress to some patients, especially children. Another disadvantage that may be an obstacle is the relatively high price and short lifespan of the devices. Even for patients with ICDs implanted, LCSD can reduce the median number of shocks per patient from 25 to 0, with a reduction of 95% [2]. Therefore, in patients with previous cardiac arrest, ICD and LCSD may complement each other by preventing sudden death and by improving quality of life through reduction in the number of shocks. Based on these considerations, both cardiologists and patients—at least in our hospital—prefer VATS-LCSD to ICD as the second choice if β-blockers were not effective or not tolerated. Whether the combination of β-blockers with VATS-LCSD can serve as first-line therapy for congenital long-QT syndrome needs to be assessed in future studies.

In conclusion, VATS-LCSD is a minimally invasive technique and has demonstrated long-term efficacy. These advantages highlight VATS-LCSD as a promising therapy in the management of congenital long-QT syndrome.

	References

Top Abstract Introduction Patients and Methods Results Comment References

 

1. Wang LX. Role of left cardiac sympathetic denervation in the management of congenital long QT syndrome J Postgrad Med 2003;49:179-181.[Medline]
2. Schwartz PJ, Priori SG, Cerrone M, et al. Left cardiac sympathetic denervation in the management of high-risk patients affected by the long-QT syndrome Circulation 2004;109:1826-1833.[Abstract/Free Full Text]
3. Moss AJ, Zareba W, Hall WJ, et al. Effectiveness and limitations of beta-blocker therapy in congenital long QT syndrome Circulation 2000;101:616-623.[Abstract/Free Full Text]
4. Li C, Hu D, Shang L, et al. Surgical left cardiac sympathetic denervation for long QT syndrome: effects on QT interval and heart rate Heart Vessels 2005;20:137-141.[Medline]
5. Wang L, Feng G. Left cardiac sympathetic denervation as the first-line therapy for congenital long QT syndrome Med Hypotheses 2004;63:438-441.[Medline]
6. Reardon PR, Matthews BD, Scarborough TK, et al. Left thoracoscopic sympathectomy and stellate ganglionectomy for treatment of the long QT syndrome Surg Endosc 2000;14:86.[Medline]
7. Li J, Wang LX, Wang J. Video-assisted thoracoscopic sympathectomy for congenital long QT syndromes Pacing Clin Electrophysiol 2003;26:870-873.[Medline]
8. Schwartz PJ, Verrier RL, Lown B. Effects of stellectomy and vagotomy on ventricular refractoriness in dogs Circ Res 1977;40:536-540.[Abstract/Free Full Text]
9. Wong CW. Stimulation of left stellate ganglion prolongs Q-T interval in patients with palmar hyperhidrosis Am J Physiol 1997;273:H1696-H1698.
10. Schwartz PJ, Malliani A. Electrical alternation of the T wave: clinical and experimental evidence of its relationship with the sympathetic nervous system and with the long QT syndrome Am Heart J 1975;89:45-50.[Medline]
11. Schwartz PJ, Snebold NG, Brown AM. Effects of unilateral cardiac sympathetic denervation on the ventricular fibrillation threshold Am J Cardiol 1976;37:1034-1040.[Medline]
12. Moss AJ, McDonald J. Unilateral cervicothoracic sympathetic ganglionectomy for the treatment of long QT interval syndrome N Engl J Med 1971;285:903-904.[Medline]
13. Liu Y-G, Shi X-Z, Liang H-P, et al. The applied anatomy of stellate ganglion and its relationship with Horner's syndrome Zhongguo Lin Chuang Jie Po Xue Za Zhi 2006;24;:167–9.
14. Epstein AE, Rosner MJ, Hageman GR, et al. Posterior left thoracic cardiac sympathectomy by surgical division of the sympathetic chain: an alternative approach to treatment of the long QT syndrome Pacing Clin Electrophysiol 1996;19:1095-1104.[Medline]
15. Thomas J, Pillay P, Mack P, Ooi LL, Nachiappan M. Video-assisted endoscope thoracic sympathectomy in the management of intractable palmar hyperhydrosis Singapore Med J 1994;35:460-463.[Medline]
16. Li CL, Hu DY, Shi XB, et al. The characteristics during exercise test in long QT syndrome patients and the effects of left cardiac sympathetic denervation Zhonghua Yi Xue Za Zhi 2005;85:2192-2195.[Medline]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Jun Wang Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Li, J. Articles by Wang, J. Search for Related Content PubMed PubMed Citation Articles by Li, J. Articles by Wang, J. Related Collections Electrophysiology - arrhythmias Related Article