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Department of Thoracic and Cardiovascular Surgery, University of Florida, PO Box 100286, HSC, Gainesville, FL 32610
(Email: klodell{at}surgery.ufl.edu).
The article by Li and colleagues [1] addresses the relatively rare but important issue of congenital long QT syndrome (LQTS). They present a retrospective review of a single-center experience using video-assisted thoracoscopic left cardiac sympathetic denervation as a minimally invasive approach for the treatment of congenital LQTS. For a 5-year period they applied this technique to 11 patients who were either intolerant of beta blockade or refused implantable cardioverter defibrillator devices, and they provided data from an average of slightly more than 3 years of follow-up. They reported complete elimination of syncope and near syncope in 7 patients, with reductions in 3 others. One patient died in the second year after surgery, yielding an 81.8% efficacy and 9.1% mortality rate.
The authors should be congratulated for their success with this technique in this difficult patient population. Criticisms of this review would include the relatively small population size, as well as the 3 patients with reduced but persistent episodes of syncope and pre-syncope recorded as treatment successes. An equally relevant statistic might be the complete responder rate of 63% (7 of 11), which still represents a tremendous success in the majority of patients with this challenging problem.
Beta blockers are a mainstay of therapy in both asymptomatic and symptomatic patients with congenital LQTS, because they reduce both syncope and sudden cardiac death. However, beta blockers are not universally well tolerated. Patients with malignant ventricular arrhythmias may also receive implantable cardioverter defibrillators, with the accompanying psychological and body image repercussions.
Left cardiac sympathetic denervation is another method to reduce cardiac events in patients with congenital LQTS. Because of the availability of other options, the main indications for this procedure are failure of medical therapy and frequent shocks with an implantable cardioverter defibrillator, despite medications. However, this procedure is more commonly used in some centers outside the United States and a few centers in the United States. The minimally invasive approach described may warrant consideration in a larger number of patients with congenital LQTS and difficulty with beta blocker therapy. A randomized controlled trial may be warranted to evaluate the use of this procedure as an alternative first-line therapy for patients with congenital LQTS.
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