HRCR Rare Diseases Article Collection
- Short QT syndrome (SQTS) is a very rare channelopathy accompanied by familial clustering and sudden cardiac death.1 It has an estimated prevalence ranging from 0.02% up to 2% in the adult population, but only 0.05% among pediatric patients.2–6 To date, 9 mutations in 6 different genes have been identified, including KCNH2, KCNQ1, KCNJ1, CACNA1C, CACNB2, and CACNA2D1. In pediatric patients, SQTS is characterized by shortening of the corrected QT interval (QTcB <316 ms, J-Tpeak cB <181 ms, and the presence of early repolarization) on the surface electrocardiogram (ECG).
- Catheter ablation is a commonly undertaken and highly effective treatment for symptomatic right ventricular outflow tract (RVOT) ventricular ectopy / ventricular tachycardia (VT).1 Despite the high chance of cure with ablation, caution is required to avoid collateral injury to coronary arteries in certain well-described locations. Anomalous coronary arteries are rare2 and may have an unfamiliar path involving the outflow tracts, posing a significantly increased risk with ablation. The approach to outflow tract ablation in a patient with an anomalous coronary artery has not been previously reported.