Serhat KOCA, Feyza AYŞENUR PAÇ, Dursun ARAS, Erdal YEKELER, Alkın YAZICIOĞLU

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

On iki yaşında kız hastaya, başka bir aile ferdinde uzun QT sendromu olması nedeniyle yapılan tarama sırasında uzun QT sendromu tanısı kondu. Beta-bloker tedavisi başlandı ve senkoplar nedeniyle intrakardiyak defibrilatör implante edildi. İzlemde ventriküler taşikardiye uygun defibrilatör şokları gözlendiği için, ventriküler taşikardiyi tetiklediği düşünülen polimorfik ventriküler ekstrasistollere ablasyon yapıldı. Bu girişime ve uygun defibrilatör şoklamalarının devam etmesine rağmen, klinik tabloya restriktif kardiyomyopati bulguları eklendi. Hastaya video yardımlı torakoskopi ile sol kardiyak sempatik denervasyon uygulandı. Cerrahi sonrası üç aylık izlem süresince, intrakardiyak defibrillatör şoklaması görülmedi. Yaşamı tehdit eden ventriküler aritmiler ile birlikte uzun QT sendromu olan hastalarda, video yardımlı torakoskopik sol kardiyak sempatik denervasyon güvenli ve etkili bir şekilde uygulanabilir

Restriktif kardiyomiyopati birlikteliği olan uzun QT sendromunda ölümcül aritmilerin tedavisinde sempatik denervasyon

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.

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