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Congenital long QT syndrome (LQTS) affects an estimated 1 in 2500 people and typically presents with syncope, seizures or sudden death. Whereas someone exhibiting marked prolongation of the QT interval with QTc exceeding 500 ms who was just externally defibrillated from torsades de pointes while swimming poses negligible diagnostic challenge as to the(More)
BACKGROUND Long QT syndrome (LQTS) typically presents with syncope, seizures, or sudden death. Patients with LQTS have been misdiagnosed with a seizure disorder or epilepsy and treated with antiepileptic drug (AED) medication. The gene, KCNH2, responsible for type 2 LQTS (LQT2), was cloned originally from the hippocampus and encodes a potassium channel(More)
Significant autonomic changes occur during seizures and may be related to sudden unexplained death in epilepsy (SUDEP). Accordingly, we performed a study to determine the prevalence of heart rate, QTc, and oximetric changes during seizures and analyzed their association with SUDEP risk factors. We analyzed 218 seizures from 76 patients. Ictal sinus(More)
Postural orthostatic tachycardia syndrome was defined in adult patients as an increase >30 beats per minute in heart rate of a symptomatic patient when moving from supine to upright position. Clinical signs may include postural tachycardia, headache, abdominal discomfort, dizziness/presyncope, nausea, and fatigue. The most common adolescent presentation(More)
AIMS Congenital or acquired QT prolongation is a risk factor for life-threatening arrhythmias. In patients with hypertrophic cardiomyopathy (HCM), the QT interval may be intrinsically prolonged. However, the prevalence, cause, and significance of QT prolongation among patients with HCM are unknown. METHODS AND RESULTS After exclusion of patients on(More)
BACKGROUND Long QT syndrome (LQTS) is a potentially lethal but highly treatable cardiac channelopathy. Treatment options include pharmacotherapy, device therapy, and left cardiac sympathetic denervation (LCSD). Here, we sought to determine the characteristics of LQTS patients who have had ≥1 LQTS-related breakthrough cardiac event (BCE) after LCSD. (More)
Orthostatic intolerance (OI) refers to a group of clinical conditions, including postural orthostatic tachycardia syndrome (POTS) and neurally mediated hypotension (NMH), in which symptoms worsen with upright posture and are ameliorated by recumbence. The main symptoms of chronic orthostatic intolerance syndromes include light-headedness, syncope or near(More)
To the Editor: Competitive sports participation for athletes with long QT syndrome (LQTS) is guided by the 36th Bethesda Conference, which recommends that patients with either (1) symptoms, (2) a corrected QT interval (QTc) greater than 470 milliseconds (males) or 480 milliseconds (females), or (3) an implantable cardioverter-defibrillator (ICD) not(More)
Sudden unexpected death in epilepsy is the sudden, unexplained, unexpected death of an individual with epilepsy in which postmortem examination does not reveal an anatomic or toxicologic cause of death. Patients with congenital long QT syndrome and catecholaminergic polymorphic ventricular tachycardia have been frequently initially diagnosed with epilepsy.(More)
Large dural arteriovenous malformations (DAVMs) accompanied by cardiac failure usually carry a poor prognosis with a high risk of morbidity and death. The authors report on the case of a male neonate with a massive DAVM who presented at birth with macrocephaly and high-output cardiac failure. The child initially underwent treatment with surgical clipping of(More)