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Short QT Syndrome (short + qt_syndrome)

Distribution by Scientific Domains

Medical Sciences	90%

Selected Abstracts

Congenital Short QT Syndrome and Implantable Cardioverter Defibrillator Treatment:

JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 12 2003
Inherent Risk for Inappropriate Shock Delivery
Introduction: A congenital short QT interval constitutes a new primary electrical abnormality associated with syncope and/or sudden cardiac death. We report on the initial use of implantable cardioverter defibrillator (ICD) therapy in patients with inherited short QT interval and discuss sensing abnormalities and detection issues. Methods and Results: In five consecutive patients from two unrelated European families who had structurally normal hearts, excessively shortened QT intervals, and a strong positive family history of sudden cardiac death, ICDs were placed for primary and secondary prevention. Mean QT intervals were 252 ± 13 ms (QTc 287 ± 13 ms). Despite normal sensing behavior during intraoperative and postoperative device testing, 3 of 5 patients experienced inappropriate shock therapies for T wave oversensing 30 ± 26 days after implantation. Programming lower sensitivities and decay delays prevented further inappropriate discharges. Conclusion: The congenital short QT syndrome constitutes a new clinical entity with an increased risk for sudden cardiac death. Currently, ICD treatment is the only therapeutic option. In patients with short QT interval and implanted ICD, increased risk for inappropriate therapy is inherent due to the detection of short-coupled and prominent T waves. Careful testing of ICD function and adaptation of sensing levels and decay delays without sacrificing correct arrhythmia detection are essential. (J Cardiovasc Electrophysiol, Vol. 14, pp. 1273-1277, December 2003) [source]

Inappropriate ICD Discharge Due to T-Wave Oversensing in a Patient with Short QT Syndrome

PACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 1 2010
YAXUN SUN M.D.
A 45-year-old man with diagnosis of short QT syndrome underwent successful implantation of dual-chamber implantable cardioverter-defibrillator (ICD) to prevent sudden cardiac death. Ten days after implantation, the patient was treated by inappropriate ICD discharges for frequent T-wave oversensing. After careful reprogramming of the ICD, the T-wave oversensing was eliminated and no T-wave oversensing or inappropriate discharge was documented during 6-month follow-up period. (PACE 2010; 113,116) [source]

T-Wave Morphology in Short QT Syndrome

ANNALS OF NONINVASIVE ELECTROCARDIOLOGY, Issue 3 2009
Olli Anttonen M.D.
Background: Short QT syndrome (SQTS) is an inherited disorder characterized by a short QT interval and vulnerability to ventricular tachyarrhythmias. The diagnostic criteria for this syndrome are not well defined, since there is uncertainty about the lowest normal limits for the corrected QT (QTc) interval. Objective: The aim of this study was to determine whether T-wave morphology parameters are abnormal in short QT subjects and whether those parameters can help in the diagnosis of SQTS. Methods and Results: We describe three families (10 patients) with short QT intervals (QTc 310 ± 32 ms). Seven subjects had suffered serious arrhythmic events and three were asymptomatic. T-wave morphology was assessed using the principal component analysis (PCA). QTc was significantly shorter and T-wave amplitude in lead V2 higher in the short QT subjects compared to healthy controls (n = 149), (P < 0.001 for both). The total cosine of the angle between the main vectors of the QRS and T-wave loops (TCRT) was markedly abnormal among the symptomatic patients with short QT syndrome (n = 7) (TCRT ,0.14 ± 0.55 vs 0.36 ± 0.51, P = 0.019). None of the three asymptomatic patients with short QT but without a history of arrhythmic events had an abnormally low TCRT. Conclusion: Our observations suggest that patients with a short QT interval and a history of arrhythmic events have abnormal T-wave loop parameters. These electrocardiogram (ECG) features may help in the diagnosis of SQTS in addition to the measurement of the duration of QT interval from the 12-lead ECG. [source]

Electrocardiographic Transmural Dispersion of Repolarization in Patients with Inherited Short QT Syndrome

ANNALS OF NONINVASIVE ELECTROCARDIOLOGY, Issue 3 2008
Olli Anttonen M.D.
Background: Short QT syndrome (SQTS) carries an increased risk for sudden cardiac death. However, only a short QT interval does not express the risk of ventricular arrhythmias. Thus, additional evaluation of the repolarization abnormality in SQTS patients is essential. In experimental models of SQTS, increased transmural dispersion of repolarization (TDR) and its electrocardiographic counterpart T-wave peak to T-wave end interval (TPE) appeared critical for induction of polymorphic ventricular tachycardia (PMVT). In a clinical study with acquired long QT syndrome patients, TPE/QT ratio > 0.28 indicated arrhythmia risk. We hypothesized that the TPE/QT ratio would be greater in SQTS patients than in control subjects. Methods and Results: We compared the behavior of the electrocardiographic TDR in three seriously symptomatic SQTS patients of unknown genotype presenting baseline QTc values <320 ms and in nine healthy age-matched control subjects. We determined QT and TPE intervals as well as TPE/QT ratio from 24-hour ECG recordings using a computer-assisted program. Diurnal average of TPE/QT ratio was 0.28 ± 0.03 in SQTS patients and 0.21 ± 0.02 in control subjects (P = 0.01). SQTS patients had also lesser capacity to change TPE intervals from steady-state conditions to abrupt maximal values than control subjects. Conclusion: SQTS patients have increased and autonomically uncontrolled electrocardiographic TDR. According to experimental SQTS models, the present results may in part explain increased vulnerability of SQTS patients to ventricular arrhythmias. [source]

T-Wave Morphology in Short QT Syndrome

Electrocardiographic Transmural Dispersion of Repolarization in Patients with Inherited Short QT Syndrome

Repolarization of the cardiac action potential.

ACTA PHYSIOLOGICA, Issue 2010
Does an increase in repolarization capacity constitute a new anti-arrhythmic principle?
Abstract The cardiac action potential can be divided into five distinct phases designated phases 0,4. The exact shape of the action potential comes about primarily as an orchestrated function of ion channels. The present review will give an overview of ion channels involved in generating the cardiac action potential with special emphasis on potassium channels involved in phase 3 repolarization. In humans, these channels are primarily Kv11.1 (hERG1), Kv7.1 (KCNQ1) and Kir2.1 (KCNJ2) being the responsible ,-subunits for conducting IKr, IKs and IK1. An account will be given about molecular components, biophysical properties, regulation, interaction with other proteins and involvement in diseases. Both loss and gain of function of these currents are associated with different arrhythmogenic diseases. The second part of this review will therefore elucidate arrhythmias and subsequently focus on newly developed chemical entities having the ability to increase the activity of IKr, IKs and IK1. An evaluation will be given addressing the possibility that this novel class of compounds have the ability to constitute a new anti-arrhythmic principle. Experimental evidence from in vitro, ex vivo and in vivo settings will be included. Furthermore, conceptual differences between the short QT syndrome and IKr activation will be accounted for. [source]

Sudden Cardiac Death and Inherited Arrhythmia Syndromes

JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 2005
ANDREA SARKOZY M.D.
Sudden cardiac death (SCD) at youth is rare and is often caused by inherited cardiac disorders. This review focuses on the genetic background of inherited primary electrical diseases, the so-called "channelopathies." Following a short clinical description of each syndrome, the recent findings in the genetics of long QT syndrome, short QT syndrome, isolated cardiac conduction defect, familial sick sinus syndrome, familial atrial fibrillation, cathecholaminergic polymorphic ventricular tachycardia, familial Wolff-Parkinson-White (WPW) syndrome, and Brugada syndrome are discussed. The currently proposed theoretical model of overlapping phenotypes in SCN5A sodium channel mutations is presented. The recent data indicate that advances in molecular genetics, experimental and clinical electrophysiology shed some light on the genetic background of primary electrical diseases. However, it is also becoming clear that the process from a mutation of a gene to the clinical presentation of a patient is currently only partially understood and extremely complex. [source]