Ann Thorac Surg 2008;86:1620-1625. doi:10.1016/j.athoracsur.2008.07.006
© 2008 The Society of Thoracic Surgeons
Original Articles: Pediatric Cardiac
Video-Assisted Thoracoscopic Cardiac Denervation: A Potential Novel Therapeutic Option for Children With Intractable Ventricular Arrhythmias
Joseph Atallah, MDa,
Francis Fynn-Thompson, MDb,
Frank Cecchin, MDa,
Daniel J. DiBardino, MDb,
Edward P. Walsh, MDa,
Charles I. Berul, MDa,*
a Department of Cardiology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts
b Department of Cardiac Surgery, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts
Accepted for publication July 1, 2008.
* Address correspondence to Dr Berul, Department of Cardiology, Children's Hospital Boston, 300 Longwood Ave, Boston, MA 02115 (Email: charles.berul{at}cardio.chboston.org).
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Abstract
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Background: Left cardiac sympathetic denervation is one of the therapeutic modalities used in the management of patients with medically refractory long QT syndrome. Traditionally, a thoracotomy or cervical incision has been used as the standard surgical approach for performing left cardiac sympathetic denervation. Video-assisted thoracoscopic surgery allows a minimally invasive technique. There is only one published series on the use of video-assisted thoracoscopic surgery for left cardiac sympathetic denervation in patients with long QT syndrome.
Methods: We performed a retrospective clinical review of pertinent medical records and report a series including 9 pediatric patients (4 long QT syndrome, 4 catecholaminergic polymorphic ventricular tachycardia, and 1 idiopathic ventricular tachycardia) who underwent a left cardiac sympathetic denervation by means of video-assisted thoracoscopic surgery.
Results: There were no severe complications, and 6 of 7 symptomatic patients with available follow-up experienced marked improvement in the first month after sympathectomy.
Conclusions: This minimally invasive procedure provides a safe novel therapeutic option for children with drug-refractory catecholaminergic polymorphic ventricular tachycardia and other catecholamine-triggered arrhythmias.
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Introduction
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Left cardiac sympathetic denervation (LCSD, also known as left cervical stellate ganglionectomy) was first described in 1971 [1]. The procedure is used for patients affected by the long QT syndrome (LQTS), and who are suboptimally controlled with standard medical therapy, usually consisting of β-adrenergic receptor blocking medications. A recent multicenter report on a large series of LQTS patients having undergone LCSD documented the efficacy of this intervention [2]. The surgical technique used in performing the LCSD varies among centers [2]. Li and colleagues [3] reported the first small series of LQTS patients undergoing LCSD using the video-assisted thoracoscopic surgery (VATS) approach.
We are aware of only one case series reporting on 3 patients with catecholaminergic polymorphic ventricular tachycardia (CPVT), a disorder of abnormal myocardial calcium homeostasis and life-threatening arrhythmias [4], who underwent a successful LCSD [5]. We now report our experience with 9 young pediatric patients who underwent video-assisted thoracoscopic LCSD, of whom 4 were diagnosed with CPVT, and another with idiopathic medically refractory ventricular tachycardia.
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Material and Methods
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A retrospective review was performed, after approval from the Committee for Clinical Investigation. The patients were identified using the cardiology database and surgical records. Data were extracted from the patients' medical charts, and then identification was removed from the record. Between 2000 and 2008, a total of 9 patients underwent LCSD at Children's Hospital Boston; all operations were performed using VATS. Of the 9 patients, 4 were diagnosed with congenital LQTS (2 of whom had Jervell and Lange-Nielsen syndrome, defined as severe QT prolongation and congenital hearing loss), 4 with CPVT, and 1 with idiopathic recalcitrant ventricular tachycardia (VT).
Surgical Technique
The LCSD procedure on all patients was performed by means of a left sided video-assisted thoracoscopic approach. After left lung isolation with a double-lumen endotracheal tube or the use of a bronchial blocker, the patient was positioned in a partial right lateral decubitus position. Three small stab incisions were made in the left chest along the midaxillary line, with one incision at the level of each of the third, fourth, and fifth intercostal spaces. The first incision was used for the camera, the second for a grasper (or lung retractor if needed), and the third one for the electrocautery hook dissector. After identifying the structures within the apex of the posterior chest wall and the heads of the ribs, the pleura was incised medial to the heads of the ribs and the sympathetic chain identified from the level of about T1 to T5 (Figs 1 and 2).
Using electrocautery, the sympathectomy involved transection of the left sympathetic chain at the level of T1 and T5 as well as the associated lateral nerves of Kuntz between those levels (Figs 1 and 2). The stellate ganglion was intentionally spared on 8 of the 9 patients to avoid iatrogenic Horner's syndrome. These 8 patients therefore received a high left thoracic sympathetic denervation, rather than a left cervical stellate ganglionectomy. A chest tube was placed in the left pleural cavity in most patients.
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Fig 1. (A) The parietal pleura is scored with electrocautery at the T5 level, revealing the underlying left sympathetic chain, which runs along the posterior chest wall just medial to the heads of the ribs. (B) The parietal pleural incision is carried up to the T1 level with electrocautery, and the sympathetic chain is divided at the T1 and the T5 level and then isolated by division of all lateral branches.
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Fig 2. (A) Illustration in the coronal plane demonstrating the right and left sympathetic chains in the thoracic paravertebral space. Labels and a caption demonstrate the stellate ganglion and the sites of the left cardiac sympathetic denervation at the levels of T1 and T5, including the lateral nerve branches. (B) Illustration in the transverse plane at the level of T5 demonstrating the video-assisted thoracoscopic surgery approach to the left sympathectomy.
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Patient Descriptions
Long QT syndrome
There were 4 patients identified with LQTS. The specific patient characteristics are listed in Table 1. Patients 1, 2, and 3 underwent placement of an epicardial dual-chamber implantable cardioverter-defibrillator (ICD) at the same time as the LCSD. Patient 4 had an ICD implanted on initial presentation at the age of 2 years, before the LCSD. Except for patient 3, the 3 other patients were considered to have failed optimized medical therapy before proceeding to LCSD.
Catecholaminergic polymorphic ventricular tachycardia
There were 4 patients identified with CPVT. The specific patient characteristics are listed in Table 1. All patients underwent placement of an ICD after the initial presentation and before LCSD. Recurrent appropriate ICD shocks despite optimized antiarrhythmic therapy was the indication for LCSD in all patients.
Patient 9 had intractable VT and had undergone three electrophysiology studies for ablation of a left-sided manifest accessory pathway and ventricular tachycardia foci. She underwent an ICD implant as well as an LCSD, resulting in only some improvement. Further control of the recurrent VT would only be achieved with high-dose labetalol and amiodarone therapy. At follow-up a year later, she was still experiencing infrequent episodes of sustained VT and ICD shocks triggered during episodes of acute illness.
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Results
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Left Cardiac Sympathetic Denervation and Video-Assisted Thoracoscopic Surgery
Left cardiac sympathetic denervation by means of VATS was performed on 9 pediatric patients, 4 with LQTS and 4 with CPVT. Four patients had an ICD implant concomitant with the LCSD. The average duration of the LCSD procedure was 35 minutes. To avoid inappropriate shocks, ICD device therapy was temporarily disabled during the use of electrocautery intraoperatively. For the 8 patients with LQTS and CPVT, the postoperative intensive care unit stay ranged from 1 to 2 days, and the total hospital stay was an average of 3 days. Routine postoperative care and pain management was appropriate for all patients. Complete clinical data for the postoperative hospital course were available for all 9 patients. All patients were on continuous cardiac rhythm monitoring (telemetry) throughout their hospital stay, and a pediatric electrophysiologist reviewed the telemetry twice daily. There were no severe surgical complications. Minor postoperative complications occurred in 2 patients. Patient 4 was noted to have prominent harlequin facial flushing, but not a Horner's syndrome, which had resolved at follow-up 5 years later. She is the only patient to have had the left stellate ganglion included in the LCSD. Patient 5 experienced sharp left arm pain that gradually improved over the course of approximately 1 year and eventually resolved.
Long QT Syndrome
Patient 2 had resolution of frequent ectopy and T-wave alternans subsequent to the LCSD, whereas in patients 1 and 4, the frequent episodes of VT completely resolved during the subsequent 2- and 5-year follow-up periods, respectively. Only patient 3 experienced an arrhythmic event 16 months after the LCSD, being his first and only ventricular fibrillation episode.
Catecholaminergic Polymorphic Ventricular Tachycardia
Patient 5 experienced a VT storm with recurrent ICD shocks in the first 8 hours after LCSD that eventually subsided. Follow-up during the subsequent 2 years revealed no further tachyarrhythmia relapses or ICD discharges, in contrast with the recurrent VT episodes with which she presented before the LCSD. In the 2 patients (patient 6 and 7) with only short-term follow-up (2 months and 1 month, respectively), there was complete resolution of their tachycardia events. Patient 8's LCSD was performed less than 1 month before the submission of the manuscript, and outpatient follow-up is not yet available.
Overall
Short-term follow-up after LCSD was available for 8 patients, 7 of whom had presented with recurrent documented arrhythmias before the LCSD. Of these 7 patients, 6 (86%) experienced a marked reduction of their life-threatening arrhythmia burden after LCSD, with 5 (71%) becoming arrhythmia free. Longer-term follow-up with an average of 30 months (range, 12 to 60 months) was available for 4 of the 8 symptomatic patients. Three patients (75%) had marked reduction in their arrhythmia burden, with 2 being arrhythmia free and 1 having had VT only during the first 8 hours after LCSD. Patient 9 with idiopathic VT had only marginal benefit from the LCSD procedure, and remained symptomatic.
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Comment
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Left Cardiac Sympathetic Denervation and Video-Assisted Thoracoscopic Surgery
In the present report, we describe our experience using minimally invasive VATS approach for performing LCSD in 9 young patients, of whom 2 had Jervell and Lange-Nielsen syndrome, 4 had CPVT, and 1 was diagnosed with idiopathic VT. There were no severe complications related to the procedure. Minor and transient postoperative complications occurred in only 2 patients. Only 1 patient experienced recurrent but transient arrhythmias in the immediate postoperative period. This novel approach to the LCSD procedure proved safe in a small pediatric cohort with life-threatening arrhythmias. Li and colleagues [3] described their experience and the safety of the VATS approach to LCSD in a small series of 4 patients (3 adults).
Long QT Syndrome
Long QT syndrome is a cardiac channelopathy with 12 different genotypes and a phenotype varying from an asymptomatic patient to sudden cardiac death as the first presentation. β-Adrenergic receptor blockers are the mainstay of therapy, and two large studies from the International LQTS Registry demonstrated their efficacy as well as their limitations [6, 7]. Recommendations for the use of ICD therapy in LQTS patients have been recently published [8], and different authors have documented the efficacy of ICDs in pediatric and adult patients [9–11].
Left cardiac sympathetic denervation is a safe and effective therapeutic modality used in the management of LQTS patients. Its use has been recommended for patients with symptoms of syncope, arrhythmias, or cardiac arrest while on β-adrenergic receptor blocker therapy [8]. A multicenter study reported on 147 patients having undergone LCSD with 99% having been symptomatic and 48% having suffered an aborted cardiac arrest before the operation. On a mean follow-up of 7.8 years after LCSD, 46% were asymptomatic and ICD shocks decreased by 95% [2]. In our cohort, all 4 patients with LQTS met recommended criteria for ICD implantation, and patients 1, 2, and 4 met criteria for LCSD. Our LCSD results compare with those of the multicenter study. For patient 3, LCSD was performed as primary prevention therapy. The latter decision was based on new data, at the time, demonstrating the increased risk of arrhythmias and sudden death in the subset of patients with Jervell and Lange-Nielsen syndrome [12, 13].
Catecholaminergic Polymorphic Ventricular Tachycardia
Catecholaminergic polymorphic ventricular tachycardia is a genetic disorder of abnormal myocardial calcium homeostasis characterized by life-threatening ventricular arrhythmias triggered during states of high sympathetic output [4]. It was first described in 1975 [14], and our understanding of its genetic and clinical characteristics has markedly evolved since then. The condition is characterized by exercise- or emotion-induced ventricular tachyarrhythmia resulting in syncope or sudden cardiac arrest. Patients with CPVT typically have structurally and functionally normal hearts, and a normal baseline electrocardiogram, including a normal QTc measurement. The VT can have a pathognomonic bidirectional morphology (Fig 3). Sporadic and familial cases have been described, with the latter exhibiting either an autosomal dominant or recessive mode of inheritance [15, 16]. Presently, the standard therapy for patients with CPVT consists of β-adrenergic blockers, and ICD implantation if necessary [4, 17].
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Fig 3. Bidirectional tachycardia during exercise testing in a patient with catecholaminergic polymorphic ventricular tachycardia.
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Three different genes have been identified and include the RyR2 gene encoding the ryanodine receptor [16, 18], the CASQ2 gene encoding the calsequestrin-2 protein [19, 20], and the ANK2 gene encoding the ankyrin-B protein [21]. In normal individuals, endogenous catecholamines activate calcium-induced calcium release by protein kinase A–mediated phosphorylation of RyR2 receptor. Dysregulation of this cascade is thought to lead to calcium leak into the myocytes and resultant malignant arrhythmias in patients with CPVT. The exact cellular molecular mechanisms in CPVT are still unclear, and different pathophysiologic mechanisms have been speculated [22].
We report our experience with 4 young patients diagnosed with CPVT and who underwent LCSD by means of a VATS technique. These patients did not suffer any significant complications related to the surgery, and 3 patients experienced a marked symptomatic improvement. Wilde and coworkers [5] also reported their experience with 3 patients with CPVT who underwent a successful LCSD and did not suffer significant complications. β-Adrenergic blockers are presently the mainstay of therapy for patients with CPVT, but persistent symptoms have been reported in 46% to 69% of patients [17, 23]. Calcium-channel blockers have also been used in conjunction with β-adrenergic blockers [24]. Recommendations for the use of ICD therapy have been published, but there are no recommendations for the use of LCSD [8]. Fatal failure of ICD therapy has been reported in a patient with CPVT [25], and we have documented failed ICD shocks in one of our patients (Fig 4). Our present understanding of the cellular and trigger mechanism in CPVT [22], in parallel with our experience with LCSD in LQTS patients [2], argues that the use of LCSD in certain patients with CPVT is a physiologically sound and safe management approach for those difficult to treat patients. The VATS procedure provides a minimally invasive approach to LCSD, which may be particularly useful for patients who are refractory or intolerant to medical therapy, as well as patients who receive excessive ICD shocks.
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Fig 4. Failed implantable cardioverter-defibrillator cardioversion with a 17-J biphasic shock in a patient with catecholaminergic polymorphic ventricular tachycardia experiencing an episode of ventricular fibrillation.
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Conclusions
This report documents the safety of the novel performance of LCSD using the VATS approach in children. We also document our limited experience with 4 patients with CPVT who underwent LCSD, with 3 experiencing significant symptomatic improvement on limited follow-up. This therapeutic modality could prove useful in the subset of patients who remain symptomatic with recurrent life-threatening arrhythmias, syncope, or frequent ICD discharges, despite standard therapy. Close follow-up of these patients is warranted to further monitor their long-term outcome.
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Acknowledgments
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This work was supported in part by the Sean Roy Johnson Memorial Fund.
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References
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Abstract
Introduction
