Introduction: Long QT interval syndrome (LQTS) is caused by an abnormality of the hearts' electrical system although the mechanical function of the heart is basically normal. Electric problems occur because of the pathological changes on the level of cellular ionic canals.
Case study: S.L. was admitted in the age of 5 months after cardiac arrest and successful resuscitation. She was resuscitated again in our institution. Ventricular tachycardia patterns which concur with TdP was registered. She had previously been treated with beta-blocators for 2 months. Rhythm normalized after an intravenous application of magnesium sulphates. We started with mexiletine and MgSO4 for 3 months but still had several of ventricular tachycardia crises of the TdP type with loss of conscience. ECG revealed LQTS as definite diagnosis (QTc was 0.54 sec). She had continously been monitored before the implantable cardioverter defibrillator was implanted (ICD) En TrustTM D 154 VCR Eurin Eucomed with epicardial electrodes transvenous SCV 6397 – 58 with a «dual chamber pacing». ICD was implanted subcutaneously in the left abdomen area and is well tolerated. This is the first infant with an ICD implanted in this way in infant age. Genetic analysis of the 10 known genes which cause LQTS interval was negative..
Conclusion: Case study presents an infant with a Long QT interval syndrome and threatening TdP incidents with lethal outcome, who had an ICD with epicardial electrodes subcutaneouslly implanted. This seems to be the youngest child with an ICD implanted in this way.