Sympathetic denervation in the treatment of fatal arrhythmias in long QT syndrome with restrictive cardiomyopathy

Serhat Koca,

doi:10.5606/tgkdc.dergisi.2016.12405

Serhat Koca¹, Feyza Ayşenur Paç¹, Alkın Yazıcıoğlu², Dursun Aras³, Erdal Yekeler²

¹Departments of Pediatric Cardiology, Türkiye Yüksek İhtisas Training and Research Hospital, Ankara, Turkey
²Departments of Thoracic Surgery, Türkiye Yüksek İhtisas Training and Research Hospital, Ankara, Turkey
³Departments of Cardiology, Türkiye Yüksek İhtisas Training and Research Hospital, Ankara, Turkey

DOI : 10.5606/tgkdc.dergisi.2016.12405

A 12-year-old female patient was diagnosed with long QT syndrome during screening performed as another member of her family had long QT syndrome. Beta-blocker therapy was initiated and an intra-cardiac defibrillator was implanted for syncopes. During follow-up, as defibrillator shocks suggesting ventricular tachycardia were detected, ablation was performed for the polymorphic ventricular extrasystoles, which were considered to induce ventricular tachycardia. Despite this intervention and ongoing proper defibrillator shocking, restrictive cardiomyopathy signs were added to the clinical presentation. The patient underwent left cardiac sympathetic denervation by the videoassisted thoracoscopy. During a three-month postoperative follow-up, no intra-cardiac defibrillator shocking occurred. Video-assisted thoracoscopic left cardiac sympathetic denervation can be safely and effectively performed in long QT patients with life-threatening ventricular arrhythmias.

Keywords : Long QT syndrome; sympathectomy; thoracoscopy

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