Objective: Congenital long QT syndrome (LQTS) provides an opportunity for neurosurgical intervention. Medication and implantable cardiac defibrillator (ICD)-refractory patients often require left cardiac sympathetic denervation (LCSD) via anterior video-assisted thoracoscopic surgery (VATS). However, this approach has major pulmonary contraindications and risks, with a common concern in children being their inability to tolerate single-lung ventilation. At Oregon Health & Science University, the authors have developed a posterior approach-extrapleural, minimally invasive, T1-5 LCSD-that minimizes this risk.
Methods: A 9-year-old girl with LQTS type III presented to the emergency department while experiencing ventricular tachycardia (VT) and ventricular fibrillation (VF) with multiple ICD firings. Medical management failed to resolve the VF/VT. VATS was attempted but could not be safely performed due to respiratory insufficiency. The patient was reintubated for dual-lung ventilation and repositioned prone. Her respiratory insufficiency resolved. Using METRx serial dilating tubes under the microscope, the left T1-5 sympathetic ganglia were sectioned and removed.
Results: Postoperatively, the patient had no episodes of VF/VT, pneumothorax, hemothorax, or Horner syndrome. With mexiletine and propranolol, she has remained largely VF/VT free, with only one VT episode during the 2-year follow-up period.
Conclusions: Minimally invasive, posterior, extrapleural, T1-5 LCSD is safe and effective for treating congenital LQTS in children, while minimizing the risks associated with VATS.
Keywords: cardiac sympathetic denervation; long QT syndrome; posterior approach; sympathectomy.