Ann Thorac Surg 2006;81:e27-e28
© 2006 The Society of Thoracic Surgeons
Case report
Nonthoracotomy Cardioverter Defibrillator Implantation in a 2-Year-Old Infant With Long QT Syndrome
Christian Schreiber, MD, PhD
a
,
*
,
Martin Kostolny, MD
a
,
Andreas Eicken, MD
b
,
Rüdiger Lange, MD, PhD
a
a Clinic for Cardiovascular Surgery, German Heart Center Munich at the Technical University Munich, Munich, Germany
b Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich at the Technical University Munich, Munich, Germany
Accepted for publication February 17, 2006.
* Address correspondence to Dr Schreiber, Clinic of Cardiovascular Surgery, German Heart Center Munich at the Technical University Munich, Lazarettstrasse 36, Munich, 80636 Germany (Email: schreiber{at}dhm.mhn.de).
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Abstract
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Implantable cardioverter defibrillator therapy is feasible and effective even in children. Unique surgical problems exist in very young patients. We report the subcutaneous placement of a defibrillation lead together with an abdominal active-can device.
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Introduction
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Even in children, defibrillation leads can often be placed transvenously [1]. However, in very small patients, placement of epicardial systems seems to be the only solution. A novel technique, which is the subcutaneous placement of a single defibrillation lead, together with an abdominal active-can device, has only been reported in another 3 patients in the current literature [2–4]. Others had used subcutaneous array leads with up to three fingers [5, 6].
A 2-year-old boy, weighing 13.4 kg, presented with long QT syndrome. At the age of 6 months, an epicardial pacemaker system was implanted at another institution with view of commencing ß-blocker therapy. Since then the patient was resuscitated after an episode of ventricular fibrillation.
At operation, the intubated patient was positioned on the right side at a 30° angle, thus exposing the spine. First the pacemaker located in the left upper abdomen was explanted and another abdominal pocket behind the right rectus abdominis muscle was created. Then a 1.5-cm transverse incision just below the scapula in the midaxillary line was performed. A slightly angled clamp was used to subcutaneously tunnel toward the spine posteriorly and superiorly. A TVL SV02 lead (St. Jude Medical Inc, Saint Paul) was slid into the tunnel and then pulled through toward the right abdominal pocket. This lead and the previously placed epicardial ventricular lead were connected to an Epic VR V-196 single-chamber implantable cardioverter defibrillator (ICD) (St. Jude Medical Inc). After confirmation of adequate sensing, defibrillation threshold testing was performed. Ventricular fibrillation was induced, and defibrillation was successfully achieved with a 20 Joule shock. At the second test, a 20 Joule shock did not terminate fibrillation, whereas a 30 Joule shock finally did terminate it. The patient was extubated on the same day and had an uneventful recovery (Fig 1). Three months after ICD implantation, pacing threshold was 1.5 V at 0.5 ms for the epicardial lead. No ventricular tachycardia was recorded and hence no ICD therapy was noted.
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Fig 1. Roentgenogram of the postoperative position of the implantable cardioverter defibrillator and the subcutaneous lead in the posteroanterior plane (left) and lateral plane (right).
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Comment
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Several small series assessed the safety, effectivity, and feasibility of ICD therapy in children [1, 7, 8]. Improvements in transvenous lead technology and device miniaturization have allowed implantations in children weighing just 20 kg [1]. However, at the time we recommended the use of passive fixation leads to avoid ventricular perforation [9]. In patients weighing less than 20 kg, usually epicardial systems are used to avoid thrombosis or vein obstruction, because any lead will occupy more than 50% of the caval vein [10]. However, epicardial system malfunction is common in long-term follow-up [11].
Despite the advancement in molecular genetic knowledge, diagnosis of congenital long QT syndrome is still based on electrocardiographic and clinical characteristics. A ß-blocker remains the mainstay treatment. For high-risk patients, the ICD offers an effective therapeutic option to reduce mortality. In the patient described, the method of subcutaneous placement of defibrillation lead proved to be fairly simple. We wish to emphasize that even though the TVL lead (St. Jude Medical Inc) we used was primarily designed for transvenous use (as also described by Kriebel and colleagues [2] in a 6-month-old patient), both its size and length were ideal in this setting. The procedure is less traumatic than an epicardial approach and yields acceptable primary defibrillation thresholds. However, the long-term clinical course with regard to defibrillation threshold in a growing child remains unknown. Therefore, further testing and a close follow-up are mandatory.
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Acknowledgments
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The authors wish to thank Jan Zdarek, from St. Jude Medical GmbH, Germany, for his assistance. The authors have performed a free and independent evaluation of this technology.
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References
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- Eicken A, Kolb C, Lange S, et al. Implantable cardioverter defibrillator (ICD) in children Int J Cardiol 2006;107:30-35.[Medline]
- Kriebel T, Ruschewski W, Paul T. Implantation of an "extracardiac" internal cardioverter defibrillator in a 6-month-old infant Z Kardiol 2005;94:415-418.[Medline]
- Luedemann M, Hund K, Stertmann W, et al. Implantable cardioverter defibrillator in a child using a single subcutaneous array lead and an abdominal active can Pacing Clin Electrophysiol 2004;27:117-119.[Medline]
- Madan N, Gaynor JW, Tanel R, et al. Single-finger subcutaneous defibrillation lead and "active can"a novel minimally invasive defibrillation configuration for implantable cardioverter-defibrillator implantation in a young child. J Thorac Cardiovasc Surg 2003;126:1657-1659.[Free Full Text]
- Berul CI, Triedman JK, Forbess J, et al. Minimally invasive cardioverter defibrillator implantation for childrenan animal model and pediatric case report. Pacing Clin Electrophysiol 2001;24:1789-1794.[Medline]
- Gradaus R, Hammel D, Kotthoff S, Bocker D. Nonthoracotomy implantable cardioverter defibrillator placement in childrenuse of subcutaneous array leads and abdominally placed implantable cardioverter defibrillators in children. J Cardiovasc Electrophysiol 2001;12:356-360.[Medline]
- Alexander ME, Cecchin F, Walsh EP, Triedman JK, Bevilacqua LM, Berul CI. Implications of implantable cardioverter defibrillator therapy in congenital heart disease and pediatrics J Cardiovasc Electrophysiol 2004;15:72-76.[Medline]
- Ten Harkel AD, Blom NA, Reimer AG, Tukkie R, Sreeram N, Bink-Boelkens MT. Implantable cardioverter defibrillator implantation in children in The Netherlands Eur J Pediatr 2005;164:436-441.[Medline]
- Schreiber C, Hausleiter J, Lange R. Ventricular perforation by a cardioverter lead in a small child Cardiol Young 2004;14:197.[Medline]
- Molina JE, Dunnigan AC, Crosson JE. Implantation of transvenous pacemakers in infants and small children Ann Thorac Surg 1995;59:689-694.[Abstract/Free Full Text]
- Brady PA, Friedman PA, Trusty JM, Grice S, Hammill SC, Stanton MS. High failure rate for an epicardial implantable cardioverter-defibrillator leadimplications for long-term follow-up of patients with an implantable cardioverter-defibrillator. J Am Coll Cardiol 1998;31:616-622.[Abstract/Free Full Text]
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