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EKG: short QT [keywords]
- Long-term follow-up of a pediatric cohort with short QT syndrome. [Journal Article, Multicenter Study]
- J Am Coll Cardiol 2013 Mar 19; 61(11):1183-91.
The purpose of this study was to define the clinical characteristics and long-term follow-up of pediatric patients with short QT syndrome (SQTS).SQTS is associated with sudden cardiac death. The clinical characteristics and long-term prognosis in young patients have not been reported.This was an international case series involving 15 centers. Patients were analyzed for electrocardiography characteristics, genotype, clinical events, Gollob score, and efficacy of medical or defibrillator (implantable cardioverter-defibrillator [ICD]) therapy. To assess the possible prognostic value of the Gollob score, we devised a modified Gollob score that excluded clinical events from the original score.Twenty-five patients 21 years of age or younger (84% males, median age: 15 years, interquartile range: 9 to 18 years) were followed up for 5.9 years (interquartile range: 4 to 7.1 years). Median corrected QT interval for heart rate was 312 ms (range: 194 to 355 ms). Symptoms occurred in 14 (56%) of 25 patients and included aborted sudden cardiac death in 6 patients (24%) and syncope in 4 patients (16%). Arrhythmias were common and included atrial fibrillation (n = 4), ventricular fibrillation (n = 6), supraventricular tachycardia (n = 1), and polymorphic ventricular tachycardia (n = 1). Sixteen patients (84%) had a familial or personal history of cardiac arrest. A gene mutation associated with SQTS was identified in 5 (24%) of 21 probands. Symptomatic patients had a higher median modified Gollob score (excluding points for clinical events) compared with asymptomatic patients (5 vs. 4, p = 0.044). Ten patients received medical treatment, mainly with quinidine. Eleven of 25 index cases underwent ICD implantation. Two patients had appropriate ICD shocks. Inappropriate ICD shocks were observed in 64% of patients.SQTS is associated with aborted sudden cardiac death among the pediatric population. Asymptomatic patients with a Gollob score of <5 remained event free, except for an isolated episode of supraventricular tachycardia, over an average 6-year follow-up. A higher modified Gollob score of 5 or more was associated with the likelihood of clinical events. Young SQTS patients have a high rate of inappropriate ICD shocks.
- Short QT interval is unreliable marker of anabolic androgenic steroid abuse in competitive athletes. [Journal Article]
- Srp Arh Celok Lek 2012 Nov-Dec; 140(11-12):711-6.
Previous animal and human studies provided the evidence that testosterone may affect ventricular repolarization by shortening of the QT interval. Synthetic derivatives of testosterone, modified to enhance its anabolic properties, are occasionally abused by some competitive athletes.We assessed whether the QT interval duration could discriminate androgenic anabolic steroids (AAS)-using strength athletes (SA) from drug-free endurance athletes (EA), by comparing 25 formulas for QT interval correction.We recruited 22 elite male athletes involved in long-term strength or endurance training and 20 sedentary controls. All elite SA reported AAS abuse, whereas EA and controls were AAS-free.AAS-using SA had markedly shorter QT-interval than AAS-free EA (348 +/- 42.3 vs. 400 +/- 34.2 ms; p < 0.001). Also, drug-free EA had a significantly longer QT-interval than sedentary persons (400 +/- 34.2 vs. 358 +/- 18.9 ms; p < 0.01). In contrast, no significant difference in the QT-interval duration was observed between AAS users and control group (348 +/- 42.3 vs. 358 +/- 18.9 ms; p = 0.394). After the QT interval was adjusted for heart rate (HR) according to 25 different formulas, only the Ashman equation yielded considerable differences among the groups that were in line with those observed before correction.Inconsistent results obtained by different correction formulas along with inability to discriminate QT (QTc) interval duration between AAS-misusing athletes and control group do not support the use of QT (QTc) interval for anti-doping purposes.
- The spectrum of acute heart failure after venlafaxine overdose. [Case Reports, Journal Article]
- Clin Toxicol (Phila) 2013 Feb; 51(2):92-5.
Venlafaxine is a bicyclic antidepressant that may be associated with severe cardiotoxicity following large overdose. The purpose of this short case series is to present different patterns of venlafaxine-related cardiotoxicity and to discuss the potential mechanisms.Between January 2010 and July 2011, four patients were admitted to an ICU with acute left ventricular failure following large venlafaxine overdoses. The age of the four female patients ranged from 35 to 65 years. None of them had no history of cardiovascular disease. The amount of venlafaxine ingested by history ranged from 3150 to 13500 mg (extended-release preparation in two cases). The peak serum venlafaxine concentration was between 2153.3 and 9950 ng/ml. Three patients died and one recovered rapidly. The initial ECG revealed only mild abnormalities in two cases. In two patients, at least one ECG recording demonstrated a widening of QRS interval. In three patients, echocardiography disclosed a left ejection fraction of 15%-18%. Two patients presented a severe serotonin syndrome, with major rhabdomyolysis. Seizures were noted in two cases, including one patient with status epilepticus. Three patients were mechanically ventilated. The causes of death were refractory hypoxemia, malignant arrhythmias, and cardiogenic shock, respectively.Severe and diffuse left ventricular dysfunction may be observed after large venlafaxine overdoses and this is not always associated with severe cardiac conduction function abnormalities. The mechanisms underlying venlafaxine-related cardiac failure with preserved normal cardiac conduction are discussed. A possible explanation may be a catecholamine-induced myocardial damage in relationship with the inhibition of norepinephrine (and dopamine) reuptake.
- Genetics of cardiac electrical disease. [Journal Article, Research Support, Non-U.S. Gov't, Review]
- Can J Cardiol 2013 Jan; 29(1):89-99.
Few tragedies compare to the sudden death of a family member. Sadly, this may represent the first sign of a familial vulnerability to such events. One common cause is an inherited cardiac arrhythmia syndrome. Sufferers are prone to premature sudden cardiac death due to altered ion channel function in the heart. Typical causes include Brugada syndrome, long QT syndrome, short QT syndrome, catecholaminergic polymorphic ventricular tachycardia, and the newly recognized early repolarization syndrome. Our knowledge of the genetic underpinnings of each of these disorders has increased markedly in recent years. Genetic screening is now a routine part of clinical care and promises more accurate diagnosis and efficient family screening. This review summarizes the diagnosis and management of each of the listed syndromes in the context of currently available genetic testing.
- The electrocardiographic changes in acute brain injury patients. [Journal Article, Research Support, Non-U.S. Gov't]
- Chin Med J (Engl) 2012 Oct; 125(19):3430-3.
Electrocardiographic (ECG) changes occurring during the course of acute brain injury (ABI) have been described frequently, but their significances remain uncertain. The present study was designed to investigate the relation of ECG abnormalities to outcome in the patients with ABI.We performed a retrospective, observational study on the ABI patients admitted to the Department of Neurosurgery of the Beijing Tiantan Hospital between December 2005 and December 2007. All the patients accepted 12-lead electrocardiographic examination within 24 hours after injury, then divided into three groups according to the Glasgow coma score (GCS). In-hospital mortality and one-month outcome assessed by the Glasgow outcome score (GOS) were investigated.Of 335 ABI patients (mean ages 32.4 years), 246 patients (73.4%) had abnormal ECGs. The most common abnormality was ST-T changes (41.5%), followed by sinus tachycardia (23.6%). ECG changes had a significant association with the severity and outcome. Logistic regression analysis showed the presence of ST-T changes (OR 2.587, 95%CI 1.009 to 6.629, P = 0.048) and QT dispersion prolongation (OR 4.656, 95%CI 1.956 to 11.082, P = 0.001) significantly associated with short outcomes.ABI can lead to myocardial damage and ECG changes had a significant association with the severity. ST-T changes and QT dispersion prolongation were the independent prognosis factors for the negative outcome of ABI patients.
- [Do you recognize short QT syndrome? A dangerous rarity!]. [News]
- MMW Fortschr Med 2012 Aug 23; 154(14):20.
- Congenital long and short QT syndromes. [Journal Article, Review]
- Cardiology 2012; 122(4):237-47.
Congenital long and short QT syndromes are familial arrhythmias characterized by derangement of repolarization and a high risk of sudden cardiac death due to ventricular tachyarrhythmias. With growing understanding of these syndromes in both the medical and lay communities, diagnostic and therapeutic difficulties are increasingly faced by health care providers. Modern genomics has determined the mechanism of arrhythmia induction in these patients, resulting in specific medical therapies and improved risk stratification. This paper reviews the common presentations, genetic etiology, basic evaluation, risk stratification, and therapeutic approach for both syndromes. Particular attention is paid to the effect of the individual syndrome on the cardiac action potential and its correlate the surface 12 lead ECG. In conclusion, patients with long and short QT syndromes are at risk for sudden death, with accurate diagnosis, risk stratification, and resulting appropriate therapy favorably altering their outcome.
- Spectrum and prevalence of mutations involving BrS1- through BrS12-susceptibility genes in a cohort of unrelated patients referred for Brugada syndrome genetic testing: implications for genetic testing. [Comparative Study, Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't]
- J Am Coll Cardiol 2012 Oct 9; 60(15):1410-8.
The aim of this study was to provide the spectrum and prevalence of mutations in the 12 Brugada syndrome (BrS)-susceptibility genes discovered to date in a single large cohort of unrelated BrS patients.BrS is a potentially lethal heritable arrhythmia syndrome diagnosed electrocardiographically by coved-type ST-segment elevation in the right precordial leads (V1 to V3; type 1 Brugada electrocardiographic [ECG] pattern) and the presence of a personal/family history of cardiac events.Using polymerase chain reaction, denaturing high-performance liquid chromatography, and DNA sequencing, comprehensive mutational analysis of BrS1- through BrS12-susceptibility genes was performed in 129 unrelated patients with possible/probable BrS (46 with clinically diagnosed BrS [ECG pattern plus personal/family history of a cardiac event] and 83 with a type 1 BrS ECG pattern only).Overall, 27 patients (21%) had a putative pathogenic mutation, absent in 1,400 Caucasian reference alleles, including 21 patients with an SCN5A mutation, 2 with a CACNB2B mutation, and 1 each with a KCNJ8 mutation, a KCND3 mutation, an SCN1Bb mutation, and an HCN4 mutation. The overall mutation yield was 23% in the type 1 BrS ECG pattern-only patients versus 17% in the clinically diagnosed BrS patients and was significantly greater among young men<20 years of age with clinically diagnosed BrS and among patients who had a prolonged PQ interval.We identified putative pathogenic mutations in ∼20% of our BrS cohort, with BrS genes 2 through 12 accounting for <5%. Importantly, the yield was similar between patients with only a type 1 BrS ECG pattern and those with clinically established BrS. The yield approaches 40% for SCN5A-mediated BrS (BrS1) when the PQ interval exceeds 200 ms. Calcium channel-mediated BrS is extremely unlikely in the absence of a short QT interval.
- [Effect of short term graded physical exercise on the level of glycemia in children and adolescents with type 1 diabetes mellitus: data of long term ECG monitoring and registration of motor activity]. [English Abstract, Journal Article]
- Kardiologiia 2012; 52(6):48-54.
Study aim was to elucidate effect of graded physical exercise on glycemia level and interval QT duration in children and adolescents with type 1 diabetes mellitus. We carried out 25-hours parallel monitoring of glycemia, ECG and physical activity in 15 children and adolescents aged 9-17 years. During monitoring these patients performed an exercise test (PWC170). We found that there were two periods of significant and prolonged lowering of glycemia: in 120-420 min and 19-21 hours after exercise. Lowering of glycemia after physical exercise was associated with prolongation of QT interval. Registration of motor activity allowed to exclude changes of glycemia due to physical activity unrelated to graded exercise.
- "Benign" early repolarization versus malignant early abnormalities: clinical-electrocardiographic distinction and genetic basis. [Journal Article, Review]
- Cardiol J 2012; 19(4):337-46.
In the great majority of cases the ECG pattern of early repolarization (ERP) is a benign phenomenon observed predominantly in teenagers, young adults, male athletes and the black race. The universally accepted criterion for its diagnosis is the presence, in at least two adjoining leads, of ≥ 1 mm or 0.1 mV ST segment elevation. In benign ERP reciprocal ST segment changes are possible only in lead aVR. In contrast, reciprocal ST segment changes can be observed in several leads in idiopathic ventricular fibrillation (IVF) and acute coronary syndrome. In benign ERP the ST segment and T wave patterns have a relative temporal stability. IVF is an entity with low prevalence, possibly familiar, and characterized by the occurrence of VF events in a young person. More frequently this occurs in male subjects without structural heart disease and with otherwise with normal ECG even using high right accessory leads and/or after ajmaline injection. Several clinical entities cause ST segment elevation include asthenic habitus, acute pericarditis, ST segment elevation myocardial infarction, Brugada syndrome, congenital short QT syndrome, and idiopathic VF. In these circumstances clinical and ECG data are most important for differential diagnosis. In IVF the modifications could be dramatic and predominantly at night during vagotonic predominance when J waves > 2 mm in amplitude. The ST/T abnormalities are dynamic, inconstant, and reversed with isoproterenol. Convex upward J waves, with horizontal/descending ST segments or "lambda-wave" ST shape are suggestive of IVF with early repolarization abnormalities. Premature ventricular contractions with very short coupling and "R on T" phenomenon are characteristics with two pattern: When originate from right ventricular outflow tract left bundle branch block morphology and from peripheral Purkinje network, left bundle branch block pattern. The inherited-familial forms are not frequent in IVF; however mutations were identified in the genes KCNJ8, DPP6, SCN5A, SCN3B, CACNA1C, CACNB2, and CACNA2D1. The management of IVF has class I indication for ICD implantation. Ablation therapy is considered additional to ICD implantation in those patients with repetitive ventricular arrhythmia. Quinidine is a highly efficient drug that prevents recurrence.