گزارش یک مورد سندرم ژرول- لانژنلسون

Long QT syndrome, which is defined by corrected QT interval longer than 0.45 seconds in men and o. 47 sec in women , could be divided into idiopathic (congenital ) and acquired forms. The idiopathic form is a familial disorder that can be associated with sensorineural deafness (Jervell and Lange- Neelson syndrome), which is transmitted with an autosomal recessive pattern. Although this syndrome could be simply diagnosed by observing QT interval in ECG, specially when history of syncope and seizure –like attacks and frequent convulsions are present, most of the time electrocardiography is interpreted as normal and the patient undergoes neurological treatments. The case under study is the first one diagnosed in the cardiology department of Imam Reza hospital as JLN Syndrome in recent years. Patient was a 12 year old girl with sensory neural deafness and recurrent syncope. For many years, the patient had been treated with antiepileptic drugs without feeling any better and with frequent syncopal episodes. After referring the patient to out- patient clinic of cardiology in Imam Reza hospital, QT interval of 520 msec was noticed, and betablocker , propranolol , with maximal tolerated dose was started. Because of high risk criteria in the patient, internal cardiac defibrilator was indicated and the patient was referred to Tehran Rajaee hospital for further management. Early diagnosis and treatment of congenital long- QT syndrome needs a highly suspicious of clinician and enough attention to ECG in a person with a history of syncopal episodes so that the physician would be able to help this group of patients, who are mostly children and teenagers, and rescue them from sudden cardiac death and complications of neurological medication.