• F S Okpokowuruk and K Bassey.
• Department of Paediatrics, University of Uyo Teaching Hospital, Uyo, Nigeria.
• Niger J Clin Pract. 2022 Jul 1; 25 (7): 1196-1198.

AbstractLong QT syndrome (LQTS) is a rare disease entity which until recently was not readily recognized as one of the causes of sudden cardiac death in children. It is a syndrome which can be congenital or acquired and is characterized by the prolongation of the QTc interval, the presence of some electrocardiographic abnormalities and other clinical parameters together with suggestive or definitive family history (Schwartz criteria). The index case is a 4-year-old female who initially presented for management on account of post native tonsillectomy hemorrhage with secondary severe anemia and associated sepsis who subsequently developed bradycardia and marked prolongation of the QTc interval on electrocardiogram. Possible factors implicated as a cause of the prolongation of the QTc include severe anemia and anesthetic drugs with a probability of an underlying genetic cause. This case highlights a rare cause of sudden cardiac death in children in our environment with the attendant difficulties in making a genetic diagnosis due to inadequate laboratory facilities.

19 keywords...

hide…