Asian Cardiovasc Thorac Ann 2000;8:52-53
© 2000 Asia Publishing EXchange Pte Ltd
Video-Assisted Modified Left Sympathectomy for Long-QT Syndrome
Weng Guo Xing, MD,
Ding Hang, MD,
Qi Juan, MD,
Xu Chun Xuan, MD
Department of Cardiovascular Surgery
Fujian Research Institute for Cardiovascular Disease
Fuzhou, Fujian, People's Republic of China
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For reprint information contact: Weng Guo Xing, MD Tel: 86 591 755 7768 Ext. 5010 Fax: 86 591 753 2356 email: gxw001{at}hotmail.com Department of Cardiovascular Surgery, Fujian Research Institute for Cardiovascular Disease, 134 East Street, Fuzhou, Fujian Province 350001, People's Republic of China.
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Abstract
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A 22-year-old female suffering from idiopathic long-QT syndrome complicated by frequent syncope, torsade-de-pointes-type ventricular tachycardia, and asthma, was successfully treated by video-assisted extensive left second and third thoracic sympathetic ganglionectomy, instead of left stellate and first thoracic ganglio-nectomy, to avoid postoperative Horner's syndrome. The QT interval was significantly shortened from 0.6 to 0.43 seconds four days after the surgery. It remained at 0.43 seconds during a 3-month follow-up with no recurrence of tachycardia or syncope.
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Introduction
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Idiopathic long-QT syndrome is an uncommon severe repolarization abnormality associated with a high frequency of syncope, ventricular tachycardia, and sudden death. We report a case of idiopathic long-QT syndrome that was successfully treated by video-assisted modified left thoracic sympathectomy.
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Case Report
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A 22-year-old woman who had suffered frequent heart palpitations and syncope since the age of 6 years, was admitted to our hospital because of a recent increase of syncopal attacks complicated by asthma since childhood. The major positive findings were a prolonged QT interval (0.6 sec) and prominent U waves, often accompanied by ventricular tachycardia and even torsade de pointes. During hospitalization, syncope occurred very frequently and beta-blockers could not be administered because of her asthma, thus a left thoracic sympathetic ganglionectomy was chosen as the primary treatment.
The patient was anesthetized, intubated, and placed in a lateral position. The anterior, middle, and posterior axillary lines of the left 4th intercostal space were chosen to create incisions of 0.5 to 1 cm for insertion of a right-angle-tip coagulating dissector, 0-degree 10-mm thoraco-scope, and a lung grasper. Another 1-cm incision was made in the 5th intercostal space between the anterior and middle axillary lines to insert a Babcock grasper. The second thoracic sympathetic ganglion was located in the intercostal space above the aortic arch and the sympathetic stem was found crossing the rib neck perpendicularly. Using these markers, pleural excision and extensive left second and third thoracic sympathectomy, including the sympathetic stem and its Kuntz branches, were performed with the coagulating dissector. The excised specimens were identified visually and sent for pathological examination to confirm excision of the sympathetic ganglions. The electrocardiogram was recorded to assess the QT interval in the operating room immediately after the sympathectomy and at frequent intervals during the postoperative follow-up.
The surgical procedure went smoothly; there was no bleeding, arrhythmia, or need for chest tube drainage. The QT interval immediately after the procedure was 0.42 sec, it was 0.58 sec 3 hours later, 0.46 sec on the 3rd postoperative day, and 0.43 sec on day 4. During 1 year of follow-up, the QT interval remained at 0.43 sec. Only one complaint of heart palpitation was noted post-operatively. However, the electrocardiogram monitor showed a normal heart rhythm when the palpitation occurred. There has been no further incidence of heart palpitation or syncope. The pathological report confirmed that the specimens were sympathetic ganglions.
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Discussion
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Idiopathic long-QT syndrome is considered to be caused by mutations in the cardiac potassium-channel or sodium-channel genes and by a congenital imbalance between the right and left thoracic sympathetic innervation, with left dominance.1,2 The characteristics of this syndrome are repolarization abnormalities, typified by a prolonged QT interval, ventricular tachycardia with torsade de pointes, frequent syncope, and sudden death.3,4 The current therapies for idiopathic long-QT syndrome include beta-blockers at a full blocking dose, which is considered the treatment of first choice and is greatly effective in reducing the frequency of syncope and mortality (from 73% percent to 6% percent according to Schwartz and colleagues2). Other treatments include a pacemaker, an implanted automatic cardioverter-defibrillator, and left stellate and first thoracic sympathectomy that is the most rational and specific therapy for most cases. Another prospective treatment is a drug to improve ionic channel dysfunction.5
Because of the contraindication to beta-blocker therapy in this case and the very frequent attacks of syncope accompanied by the deadly torsade de pointes, im-plantation of a pacemaker or automatic cardioverter-defibrillator was considered to be inadequate. In addition, such devices are too expensive for some Chinese patients such as this farmer's daughter. Therefore, a video-assisted left thoracic sympathetic ganglionectomy was performed. In the traditional method of sympathectomy, the gang-lionectomy includes the left stellate and first thoracic ganglion, which often results in Horner's syndrome. This was considered to be unacceptable in a 22-year-old. Thus, a modified left sympathectomy, extensively excising the second and third left thoracic sympathetic ganglions, was designed to achieve a restoration of balance between the right and left thoracic sympathetic innervation, shortening the prolonged QT interval and eliminating the deadly torsade de pointes and syncope. The surgical results demonstrated that the modified procedure was able to achieve the same excellent clinical results and there was no Horner's syndrome or other major complication. Schwartz and colleagues6 reported that left thoracic sympathectomy can raise the ventricular fibrillation threshold and prevent sudden death from arrhythmia. However, it should be kept in mind that atropine and any other agent that may induce ventricular arrhythmias in patients with long-QT syndrome should not be ad-ministered perioperatively. Furthermore, as the modified procedure was performed by a video-assisted thoraco-scopic technique, the surgical trauma was minimized. This method of treatment is recommended for further clinical application and study.
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References
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Zareba W, Moss AJ, Schwartz PJ, Vincent GM, Robinson JL, Priori SG, et al. Influence of genotype on the clinical course of the long-QT syndrome. International Long-QT Syndrome Registry Research Group. N Engl J Med 1998; 339:960–5.[Abstract/Free Full Text]
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Schwartz PJ, Periti M, Malliani A. The long Q-T syndrome. Am Heart J 1975;89:378–90.[Medline]
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Schwartz PJ. The idiopathic long-QT syndrome. Ann Intern Med 1983;99:561–2.
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Moss AJ. Management of patients with the hereditary long-QT syndrome. J Cardiovasc Electrophysiol 1998; 9:668–74.[Medline]
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Moss AJ. Clinical management of patients with the long-QT syndrome: drugs, devices, and gene-specific therapy. Pacing Clin Electrophysiol 1997;20:2058–60.[Medline]
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Schwartz PJ, Snebold NG, Brown AM. Effects of unilateral cardiac sympathetic denervation on the ventricular fibrillation threshold. Am J Cardiol 1976;37:1034–40.[Medline]
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Abstract
Introduction
