BACKGROUND The 2005 Bethesda Conference Guidelines advise patients with long QT syndrome against competitive sports. We assessed cardiac event rates during competitive and recreational sports, and daily activities among treated long QT syndrome patients. METHODS AND RESULTS Long QT syndrome patients aged ≥4 years treated with anti-adrenergic therapy were included. Demographics included mechan...

Subarachnoid hemorrhage (SAH) is a neurologic emergency associated with high mortality rate. Polymorphic ventricular tachycardia (VT) is a rare arrhythmia. It can occur in any setting of a long QT interval and bradycardia. This may result from a cardiomyopathy (both ischemic and non-ischemic), acute coronary ischemia, congenital long QT syndrome, electrolyte disturbances and cerebrovascular dis...

T wave alternans (TWA), an alteration of the amplitude and morphology of the T wave that occurs every-other-beat, is emerging as an important new marker for identifying patients at risk of developing malignant ventricular arrhythmias. {Rosenbaum, Jackson, et. Al 1994} It is undergoing intense investigation in a number of areas where patients are thought to be at higher risk for Sudden Cardiac D...

The occurrence of takotsubo cardiomyopathy in a patient with congenital long QT syndrome has rarely been described. This case report discusses the occurrence of a clinically overt takotsubo cardiomyopathy accompanied by congenital long QT syndrome type 1 in a female patient.

Timothy syndrome, long QT syndrome type 8, is highly malignant with ventricular tachyarrhythmia. A 30-month-old boy had sudden cardiac arrest during anesthesia induction before plastic surgery for bilateral cutaneous syndactyly. After successful resuscitation, prolonged QT interval (QTc, 0.58-0.60 sec) and T-wave alternans were found in his electrocardiogram. Starting β-blocker to prevent furth...

Infective endocarditis in hypertrophic obstructive cardiomyopathy is uncommon. We present a case of extensive infective endocarditis in a female patient with mild hypertrophic obstructive cardiomyopathy and long QT syndrome. Upon a definite diagnosis, an urgent operation was performed successfully, and the patient had an uneventful postoperative course. The clinical features and the possible me...

Antzelevitch C. Role of spatial dispersion of repolarization in inherited and acquired sudden cardiac death syndromes. Am J Physiol Heart Circ Physiol 293: H2024–H2038, 2007. First published June 22, 2007; doi:10.1152/ajpheart.00355.2007.— This review examines the role of spatial electrical heterogeneity within the ventricular myocardium on the function of the heart in health and disease. The c...

This review examines the role of spatial electrical heterogeneity within the ventricular myocardium on the function of the heart in health and disease. The cellular basis for transmural dispersion of repolarization (TDR) is reviewed, and the hypothesis that amplification of spatial dispersion of repolarization underlies the development of life-threatening ventricular arrhythmias associated with...

BACKGROUND A paradoxical increase in the uncorrected QT interval during infusion of low-dose epinephrine appears pathognomonic for type 1 long-QT syndrome (LQT1). We sought to determine the diagnostic accuracy of this response among patients referred for clinical evaluation of congenital long-QT syndrome (LQTS). METHODS AND RESULTS From 1999 to 2002, 147 genotyped patients (125 untreated and ...