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LQT Syndrome

Decent Essays
Chapter 1 - General introduction

Due to great progress in genetic testing during the last decade, there is an increasing awareness of congenital heart diseases that give a higher risk of sudden cardiac death (SCD). A good example of such heart disease is the congenital Long QT Syndrome (LQTS). This disorder is a leading cause of SCD in the non-adult population.1 Ion channels responsible for the ventricular repolarization are mutated, causing a prolongation of the QT-interval on ECG.2 This aberrant repolarization of the heart gives a risk of the occurrence of ventricular arrhythmias, called torsades de pointes. These arrhythmias are mainly precipitated by emotional or physical stress2 and cause symptoms such as syncope or cardiac arrest. The prevalence of LQTS is around 1:2000.3 The most common involved LQTS genes are KCNQ1, KCNH2 and SCN5A, resulting in the subtypes LQT1, LQT2 and LQT3 respectively.3 Each mutation has its own clinical manifestations and its own specific management. The diagnosis for LQTS is established by the use of the Schwartz score.4 This scoring
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These include infection, occurrence of inappropriate shocks and the potential of inducing, instead of terminating ventricular arrhythmias.10, 11 On top of this, younger patients need more device replacements. Due to their active life style they are more likely for lead fractures, causing more frequently inappropriate shocks.12 Besides these complications, patients are experiencing fear for (in)appropriate discharge and they are disqualified for competitive sports participation.13 All in all, this raises the question whether ICD-treatment in young people is the most qualified one. This dilemma is also postulated in the following
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