Perioperative anesthetic management and procedural sedation in a child with type 1 congenital long QT Syndrome undergoing serial procedures: a case report

BACKGROUND: Congenital long QT syndromes (cLQTS) are inherited cardiac channelopathies that predispose pediatric patients to ventricular arrhythmias, particularly Torsades de Pointes (TdP), during the perioperative period. Data regarding repeated anesthetic and sedation exposures in this high-risk population remain limited. CASE PRESENTATION: We present an 8-year-old boy with genetically confirmed LQT1 and multiple systemic comorbidities who underwent five general anesthetics and four procedural sedations within a four-month timeframe. Management focused on preserving hemodynamic stability, attenuating autonomic fluctuations, minimizing drug-induced prolongation of cardiac repolarization, and ensuring immediate availability of rescue resources. Anesthesia was maintained via balanced volatile or intravenous regimens, while procedural sedation employed dexmedetomidine-midazolam based anxiolysis. Across all nine interventions, no TdP, malignant ventricular tachyarrhythmia, treatment-requiring bradycardia, or cardiac arrest was documented. CONCLUSIONS: This single-patient experience describes repeated general anesthesia and procedural sedation in a child with LQT1 without documented adverse cardiac events.