Author/Editor		Dolinšek, J; Vesel, S; Mazić, U; Podnar, T
Title		Sindrom dolgega intervala QT
Translated title		Long QT syndrome
Type		članek
Source		Slov Pediatr
Vol. and No.		Letnik 9, št. 1
Publication year		2002
Volume		str. 4-11
Language		slo
Abstract		Long QT syndrome (LQTS) is caused by abnormal electrophysiology of the heart, manifesting with typical ECG changes and life - threatening arrhythmias. Two hypotheses are trying to explain the pathogenesis of the LQTS: sympathetic imbalance and intracardiac abnormality hypothesis. Diagnosis of LQTS is based upon diagnostic criteria including clinical findings, family history and ECG changes. A point system enables us to separate patients into three categories with respect to the probability of the disease. Of particular importance are patients having acquired prolongation of QT interval mainly caused by administration of certain medications or electrolyte imbalance in predisposed patients. Beta-blockers are essential therapy for patients with LQTS. Therapy may also include implantation of cardiac pacemaker or cardioverter - defibrilator, and in rare patients left cardiac sympathetic denervation. Newer medications, acting on ion channels, represent a promising tool for the management of LQTS patients in the future.
Summary		Sindrom dolgega intervala QT (LQTS) je odraz motenih elektrofizioloških funkcij srca, kar se manifestira z značilnimi spremembami v elektrokardiogramu in življenjsko nevarnimi ventrikularnimi tahikardijami. Patogenezo LQTS skušata pojasniti dve hipotezi: hipoteza neuravnoteženega delovanja simpatičnega živčevja in hipoteza intrakardialnih abnormalnosti. V prid drugi hipotezi govori odkritje spremenjenih genov pri bolnikih z LQTS, ki kodirajo specifične ionske kanale. Diagnoza LQTS temelji na klinični sliki, družinski anamnezi in elektrokardiografskih spremembah. Poseben problem predstavljajo bolniki s pridobljenim podaljšanjem intervala QT, ki je najpogosteje posledica delovanja različnih zdravil ali motenj v ravnotežju elektrolitov pri predisponiranih bolnikih. Zdravila izbire pri bolnikih z LQTS so beta-blokatorji. Pri zdravljenju uporabljamo tudi srčne spodbujevalnike, kardioverterske-defibrilatorje in levo cervikotorakalno simpatično gangliektomijo. Novejša zdravila, ki delujejo na specifične ionskih kanale, napovedujejo bolnikom boljšo prognozo.
Descriptors		LONG QT SYNDROME TACHYCARDIA, VENTRICULAR DEATH, SUDDEN, CARDIAC ELECTROCARDIOGRAPHY CHILD