Electrocardiogram screening of deaf children for long QT syndrome: are we following UK national guidelines?

2010 ◽  
Vol 125 (4) ◽  
pp. 354-356 ◽  
Author(s):  
S L Kang ◽  
C Jackson ◽  
W Kelsall
Keyword(s):  
Cochlear Implant ◽  
Long Qt Syndrome ◽  
Qt Interval ◽  
Deaf Children ◽  
National Guidelines ◽  
Long Qt ◽  
Local Practice ◽  
Qt Intervals ◽  
Centre Database ◽  
Qt Syndrome

AbstractIntroduction:Jervell–Lange-Nielsen syndrome is characterised by congenital deafness and a long QT interval on electrocardiography.Aim:(1) To survey UK national practice regarding electrocardiography screening of deaf children referred to cochlear implant centres, performed to evaluate for prolonged QT interval as recommended by national guidelines, and (2) to review local practice.Methods:Data were collected via a questionnaire sent to all UK cochlear implant centres, and via review of the medical records of a local cochlear implant centre database.Results:Eight (42 per cent) of the 19 cochlear implant centres surveyed performed electrocardiographic screening. Thirteen cases of long QT syndrome were reported in seven centres, with two related deaths. In our local cochlear implant centre, 14 (7.1 per cent) of 193 children had abnormal electrocardiograms; one definite long QT syndrome case and 13 borderline cases were identified.Conclusion:Despite clear national guidelines for electrocardiographic screening of deaf children, there is wide variation in practice. Our local practice of performing investigations, including electrocardiography, during magnetic resonance imaging sedation has been very successful. Electrocardiograms should be reviewed by trained clinicians, and corrected QT intervals should be calculated manually.

scholarly journals Response of the QT interval to mental and physical stress in types LQT1 and LQT2 of the long QT syndrome

Heart ◽  
2001 ◽  
Vol 86 (1) ◽  
pp. 39-44
Author(s):  
K J Paavonen ◽  
H Swan ◽  
K Piippo ◽  
L Hokkanen ◽  
P Laitinen ◽  
...  
Keyword(s):  
Heart Rate ◽  
Serum Potassium ◽  
Long Qt Syndrome ◽  
Qt Interval ◽  
Mental Stress ◽  
Physical Stress ◽  
Exercise Stress ◽  
Long Qt ◽  
Qt Intervals ◽  
Qt Syndrome

OBJECTIVETo study and compare the effects of mental and physical stress on long QT syndrome (LQTS) patients.DESIGNCase–control study.MAIN OUTCOME MEASURESQT intervals were measured from lead V3. Serum potassium and plasma catecholamine concentrations were also monitored.PATIENTS16 patients with type 1 LQTS (LQT1), 14 with type 2 LQTS (LQT2), both groups asymptomatic, and 14 healthy control subjects.INTERVENTIONSThree types of mental stress tests and a submaximal exercise stress test.RESULTSHeart rate responses to mental stress and exercise were similar in all groups. During mental stress, the mean QT interval shortened to a similar extent in controls (–29 ms), LQT1 patients (–34 ms), and LQT2 patients (–30 ms). During exercise, the corresponding QT adaptation to exercise stress was more pronounced (p < 0.01) in healthy controls (–47 ms) than in LQT1 (–38 ms) or LQT2 patients (–38 ms). During exercise changes in serum potassium concentrations were correlated to changes in QT intervals in controls, but not in LQTS patients. LQT1 and LQT2 patients did not differ in serum potassium, catecholamine or heart rate responses to mental or physical stress.CONCLUSIONSQT adaptation to mental and exercise stress in healthy people and in patients with LQTS is different. In healthy people QT adaptation is more sensitive to physical than to mental stress while no such diverging pattern was seen in asymptomatic LQTS patients.

Abstract 14358: Treadmill Exercise Testing Improves Diagnostic Accuracy in Children With Concealed Congenital Long QT Syndrome

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Trisha Patel ◽  
Stanley Kamande ◽  
Elizabeth Jarosz ◽  
James Bost ◽  
Sridhar hanumanthaiah ◽  
...  
Keyword(s):  
Exercise Testing ◽  
Long Qt Syndrome ◽  
Stress Testing ◽  
Normal Population ◽  
Recovery Phase ◽  
Exercise Stress ◽  
Long Qt ◽  
Qt Intervals ◽  
Prolonged Qt ◽  
Qt Syndrome

Introduction: Resting electrocardiogram (ECG) identification of long QT syndrome (LQTS) has limitations. Uncertainty exists on how to classify patients with borderline prolonged QT intervals. We tested if exercise testing could help serve as a guide for which children with borderline prolonged QT intervals may be gene positive for LQTS. Methods: Pediatric patients (n=139) were divided into three groups: Controls (n=76), gene positive LQTS with borderline QTc (n=21), and gene negative patients with borderline QTc (n=42). Borderline QTc was defined between 440 to 470 (male) and 440 to 480 (female) msec. ECGs were recorded while supine, sitting, and standing. Patients then underwent treadmill stress testing using the Bruce protocol followed by a 9-minute recovery phase. Statistical analysis was completed to compare the QTc intervals amongst all three of the groups using t-test, ANOVA, and the Youden method to calculate sensitivity and specificity cut points. Results: Supine resting QTc, age, and Schwartz score for the three groups were: 1) Gene positive: 446 ± 23 msec, 12.4 ± 3.4 yo, 3.2 ± 1.8; 2) Gene negative: 445 ± 20 msec, 12.1 ± 2.8 yo, 2.0 ± 1.2; and 3) Control: 400 ± 24 msec, 15.0 ± 3 yo. The three groups could be differentiated by their QTc response at two time points: standing and recovery phase at six minutes. Standing QTc ≥ 460 msec differentiated borderline prolonged QTc patients (Gene positive and Gene negative) from controls with a specificity of 90% for gene positive versus control and 83% for gene negative versus control. A late recovery QTc ≥ 480 msec at minute six distinguished Gene positive from Gene negative patients with a specificity of >97%. Conclusions: Exercise stress testing can be useful to identify Gene positive borderline LQTS from a normal population and Gene negative borderline QTc patients, allowing for increased cost effectiveness by selectively gene testing a higher risk group of patients with borderline QTc intervals and intermediate Schwartz scores.

Reproducibility of corrected QT interval in pediatric genotyped long QT syndrome

2016 ◽  
Vol 58 (11) ◽  
pp. 1246-1248
Author(s):  
Yoshiharu Ogawa ◽  
Toshikatsu Tanaka ◽  
Sachiko Kido
Keyword(s):  
Long Qt Syndrome ◽  
Qt Interval ◽  
Long Qt ◽  
Corrected Qt Interval ◽  
Qt Syndrome

scholarly journals Prolonged QTc: "Mind the Gap"

2014 ◽  
Vol 2 (1) ◽  
pp. 44-45
Author(s):  
Ahmad Mursel Anam ◽  
Raihan Rabbani ◽  
Farzana Shumy ◽  
M Mufizul Islam Polash ◽  
M Motiul Islam ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Long Qt Syndrome ◽  
Qt Interval ◽  
Torsades De Pointes ◽  
Junior Doctors ◽  
Long Qt ◽  
Acquired Long Qt Syndrome ◽  
Prolonged Qt Interval ◽  
Prolonged Qt ◽  
Qt Syndrome

We report a case of drug induced torsades de pointes, following acquired long QT syndrome. The patient got admitted for shock with acute abdomen. The initial prolonged QT-interval was missed, and a torsadogenic drug was introduced post-operatively. Patient developed torsades de pointes followed by cardiac arrest. She was managed well and discharged without complications. The clinical manifestations of long QT syndromes, syncope or cardiac arrest, result from torsades de pointes. As syncope or cardiac arrest have more common differential diagnoses, even the symptomatic long QT syndrome are commonly missed or misdiagnosed. In acquired long QT syndrome with no prior suggestive feature, it is not impossible to miss the prolonged QT-interval on the ECG tracing. We share our experience so that the clinicians, especially the junior doctors, will be more alert on checking the QT-interval even in asymptomatic patients. DOI: http://dx.doi.org/10.3329/bccj.v2i1.19970 Bangladesh Crit Care J March 2014; 2 (1): 44-45

scholarly journals Drug-induced long qt syndrome

2020 ◽  
Vol 27 (3) ◽  
pp. 42-52
Author(s):  
G. A. Golovina ◽  
K. V. Zaphiraki ◽  
E. D. Kosmacheva
Keyword(s):  
Long Qt Syndrome ◽  
Qt Interval ◽  
Diagnosis And Treatment ◽  
Fatal Complication ◽  
Long Qt ◽  
Drug Induced ◽  
Long Qt Interval ◽  
Qt Syndrome

In this review drug-induced long QT interval syndrome is described. The authors discuss approaches for the prevention, diagnosis, and treatment of this potentially fatal complication.

Effect of age and overweight on the QT interval and the prevalence of long QT syndrome in children

2002 ◽  
Vol 89 (4) ◽  
pp. 395-398 ◽  
Author(s):  
Toshiro Fukushige ◽  
Masao Yoshinaga ◽  
Atsushi Shimago ◽  
Junichiro Nishi ◽  
Yukiharu Kono ◽  
...  
Keyword(s):  
Long Qt Syndrome ◽  
Qt Interval ◽  
Long Qt ◽  
Qt Syndrome ◽  
Effect Of Age

Abstract 1778: Electrocardiographic and Genetic Screening for Long QT Syndrome: Results from a Prospective Study on 44,596 Neonates

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Marco Stramba-Badiale ◽  
Lia Crotti ◽  
Karine Goulene ◽  
Matteo Pedrazzini ◽  
Savina Mannarino ◽  
...  
Keyword(s):  
Sudden Death ◽  
Genetic Analysis ◽  
Prospective Study ◽  
Long Qt Syndrome ◽  
Qt Interval ◽  
Family Members ◽  
Long Qt ◽  
Prolonged Qt ◽  
Qt Syndrome ◽  
A Prospective Study

Background. The long QT syndrome (LQTS), a leading cause of sudden death under 20 years of age, is due to mutations in genes which encode ion channels involved in the control of ventricular repolarization. In a prospective study on 34,000 neonates we found that a prolonged QT interval was associated with a 41 times greater risk for sudden infant death syndrome (SIDS) and, recently, in a case-control study on 201 cases of SIDS we found disease-causing LQTS mutations in 9.5% of the victims. Based on these results the Italian Ministry of Health is considering the possibility of introducing in the National Health Service an electrocardiographic (ECG) screening program in the first month of life to identify infants affected by LQTS. A realistic assessment of the prevalence of infants with LQTS becomes necessary. Methods. An ECG was recorded in the first month of life in 44,596 neonates. The QT interval was measured and corrected for heart rate according to the Bazett’s formula (QTc). In the neonates with a markedly prolonged QT (QTc ≥ 470 msec) molecular screening of the LQTS genes was performed. Results. A QTc between 440 and 470 msec was observed in 611 neonates (1.4%). A QTc ≥ 470 ms was found in 31 neonates (0.07%). Genetic analysis was performed in 28/31 (90%) neonates and LQTS mutations were identified in 14 of them (50%): 8 were LQT1, 4 LQT2 and 2 LQT3. Besides one de novo mutation, all other cases were familial and genetic analysis identified additional family members (37/72, 51%) affected by LQTS who had not been previously diagnosed. Within these 28 infants QTc was longer in the positively genotyped neonates (493±22 vs 479±6 ms, p=0.037) and a LQTS mutation was identified in all the neonates (n=5) with a QTc > 496 ms. Conclusions. An ECG performed in the first month of life, with genetic analysis in selected cases, allows early diagnosis of infants with sporadic and familial forms of LQTS, thus leading to institution of effective therapies aimed at preventing sudden death either in infancy or later on in life, not only in the neonates but also in their affected family members. This study also provides a first data-based estimate of LQTS prevalence, likely to be between 1/3,000 and 1/2,500 live births.

scholarly journals Mexiletine shortens the QT interval in a pedigree of KCNH2 related long QT syndrome

2020 ◽  
Vol 36 (1) ◽  
pp. 193-196 ◽  
Author(s):  
Taishi Fujisawa ◽  
Yoshiyasu Aizawa ◽  
Yoshinori Katsumata ◽  
Kensuke Kimura ◽  
Kenji Hashimoto ◽  
...  
Keyword(s):  
Long Qt Syndrome ◽  
Qt Interval ◽  
Long Qt ◽  
Qt Syndrome
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