Electrocardiogram screening of deaf children for long QT syndrome: An Egyptian experience.

BACKGROUND: Jervell and Lange-Nielsen syndrome is an autosomal recessive form of long QT syndrome (LQTS), clinically manifested by long QT interval and bilateral sensorineural hearing loss (SNHL) with the highest prevalence in Norway and Sweden. No data are available about the prevalence of such syndrome in Egypt.

OBJECTIVES: This study aimed to assess by electrocardiogram (ECG) the prevalence of LQTS among Egyptian children with SNHL.

METHODS: One thousand and twelve patients, aged ≤ 10 years (mean age 5.8 ± 2.6), were included in this study, 578 male patients (57%) and 434 female patients (43%). A 12-lead ECG was recorded for all patients and the corrected QT interval (QTc) was calculated by Bazett's formula. The probability of LQTS was evaluated by Schwartz criteria and laboratory investigations were done on all patients with long QT interval.

RESULTS: In the current study, the mean QTc interval was 411.7 ± 25.3 ms (range 343-675 ms). Twenty-one patients (2.1 %) had probable LQTS; of these, 11 patients had intermediate probability (Schwartz score 1.5-3 points) and 10 patients had high probability (Schwartz score ≥ 3.5 points).

CONCLUSION: This study shows that 2.1% of Egyptian children with SNHL in a tertiary care setting have LQTS.