Case of Williams-Beuren syndrome presenting with recurrent atrioventricular nodal re-entrant tachycardia managed with electrophysiological study and radiofrequency ablation

Objective: Williams-Beuren syndrome (WBS) carries markedly elevated sudden cardiac death risk primarily from structural cardiovascular anomalies and QTc prolongation.Supraventricular tachycardias, particularly atrioventricular nodal re-entrant tachycardia (AVNRT), remain unreported.Case presentation: A young adult in twenties with genetically confirmed WBS (7q11.23 deletion) and supravalvular aortic stenosis (SVAS) presented with recurrent palpitations.Electrocardiogram documented narrow-complex tachycardia (180 bpm, short RP interval <70 ms) diagnostic of typical slow-fast AVNRT.Evaluation prompted electrophysiological study that confirmed recurrent paroxysmal supraventricular tachycardia, typical slow fast AVNRT.Radiofrequency ablation of the slow pathway was achieved without complications.Conclusion: This represents the first documented electrophysiological study confirmed AVNRT in WBS.Early electrophysiological study and catheter ablation provide safe, curative therapy even amid structural heart disease and coronary anomaly risks.