Work

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

Year: 2026

Type: article

Abstract: 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.

Source: Heart Vessels and Transplantation

DOI: 10.24969/hvt.2026.626

Authors: Chetan Bhole, Atharva Telhande, Sanket Bari

Institutions: Dr. D. Y. Patil Medical College, Hospital and Research Centre, NKP Salve Institute of Medical Sciences and Lata Mangeshkar Hospital

Language: English

Topic: Cardiac Arrhythmias and Treatments

Subfield: Cardiology and Cardiovascular Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $350