Zespół wydłużonego odstępu QT u 13-letniej dziewczynki z nawracającymi zasłabnięciami o podłożu ortostatycznym – opis przypadku

*Zbigniew Krenc^{1, 2}, Sylwia Kowalczyk-Jurgiel³

Summary
Syncope occurs commonly in children and adolescents. Such an event may result from a wide variety of causes, ranging from benign conditions to life-threatening diseases.
Cardiogenic syncope are rare causes of loss of consciousness and indicate on serious pathology in the cardiovascular system, such as cardiomyopathies, e.g. hypertrophic cardiomyopathy or arrhythmogenic right ventricular cardiomyopathy, and ion channelopathies, e.g. Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia or long QT syndrome.
Congenital long QT syndrome is a genetic condition characterized by a prolonged QT interval on the surface ECGand is associated with a high risk of sudden cardiac death due to ventricular tachyarrhythmias. Mutations within 13 identified genes cause a variety of channelopathies affecting myocardial repolarization, resulting in QT prolongation.
Physical activity is not per se the cause of the enhanced mortality in long QT syndrome but it rather acts as a trigger of cardiac arrest. So, pre-participation cardiovascular evaluation, including a resting 12-lead ECG, allow to detect still asymptomatic athletes with life-threatening heart diseases to protect them from sudden cardiac death.
Our paper presents a case of 13-year-old female athlete with clinically asymptomatic long QT syndrome and recurrent orthostatic presyncope. Long QT syndrome was diagnosed in patient’s father too.

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