Syndrome de Romano-Ward familial. À propos de deux nouvelles observations

Abstract

Congenital long QT syndrome is rare, usually revealed by bouts of syncopal attacks secondary to effort or strong emotions, and more rarely by atypical epileptic crisis. We report a family history of two boys whose mother and grandmother both died suddenly a few days after delivery. The oldest child was 10 years old when admitted to hospital for recurrent loss of consciousness. Neurological examination and biological assays were normal; electrocardiography (ECG) revealed a prolonged QT interval of 0.59 seconds and episodes of torsades de pointe on the 24 hour ECG recording. The inefficacy of beta blocker treatment alone led to the implantation of a pacemaker; no recurrence has occurred since. The family investigation permitted to recognize the same syndrome in his asymptomatic 8-year-old brother for whom a prophylactic treatment was started. Both cases remind us of the necessity to carry out systematically an ECG in every child seen for unexplained malaise related or not to stress or for an atypical epileptic crisis. This is the only way for an early diagnosis on which the entire prognosis depends.