Double anomalies: Brugada syndrome presenting with a persistent left superior vena cava

Objective: Rare co-existance of disease or pathology

Background: The presentation of Brugada syndrome (BrS) with a persistent left superior vena cava (PLSVC) is expected to be a rare entity. It is unknown if this venous anomaly is linked to the arrhythmogenesis seen in BrS, or it is coincidental. This case describes a clinical presentation of the 2, in tandem, and displays the anomaly in association with BrS.

Case Report: A 54-year-old female presented to the Emergency Department with non-prodromal syncope. This was on a background of a number of similar episodes in the past, and a current suspected viral illness comprising fever and diarrhea. Her resting electrocardiogram was suggestive of BrS. The later was confirmed with an ajmaline provocation test after ECG normalization in the subsequent 24 hours post admission. Pre-intracardiac defibrillator (ICD) procedure imaging displayed the PLSVC. An ICD was implanted, and the advancement of the guidewires displayed the venous anomaly. Post-procedure echocardiography confirmed appropriate positioning of the leads. The patient recovered well and is currently symptom free.

Conclusions: PLSVC presenting with BrS is a rare occurrence. It is unknown whether or not the PLSVC and BrS are linked in their presentation, or merely a coincidence