Diagnostic accuracy of screening electrocardiograms in long QT syndrome I.

@article{Miller2001DiagnosticAO,
  title={Diagnostic accuracy of screening electrocardiograms in long QT syndrome I.},
  author={M D Miller and Porter Cb and Michael J. Ackerman},
  journal={Pediatrics},
  year={2001},
  volume={108 1},
  pages={
          8-12
        }
}
OBJECTIVE Inherited long QT syndrome (LQTS) may present with syncope, seizures, and/or sudden death as a result of ventricular tachyarrhythmias. Identification of family members who are at risk because they harbor the genetic substrate for LQTS is critical. Presently, such identification relies on the 12-lead electrocardiogram (ECG). The purpose of this study was to evaluate the efficacy of the automated ECG as a screening tool for LQTS. METHOD Molecular testing of a proband and 22 additional… 
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Electrocardiographic risk stratification in families with congenital long QT syndrome.
TLDR
QTc is the best diagnostic and prognostic ECG parameter in LQTS families and a single measurement should be obtained in lead II if measurable and then in left precordial leads (preferably V5) as a second choice.
Two automatic QT algorithms compared with manual measurement in identification of long QT syndrome.
Electrocardiogram Screening for Disorders That Cause Sudden Cardiac Death in Asymptomatic Children: A Meta-analysis
TLDR
Results provide an evidence base for evaluating pediatric screening for these disorders and show ECG, alone or with ECHO, was a sensitive test for mass screening and negative predictive value was high, but positive predictive value and false-positive rates varied.
Vectorcardiographic Recordings of the Q-T Interval in a Pediatric Long Q-T Syndrome Population
TLDR
The VCG automatic measurement of QTc seems to be a better predictor of LQTS than automatic measurement and interpretation of 12-lead ECG.
Prolonged QT Interval Diagnosis Suppression by a Widely Used Computerized ECG Analysis System
TLDR
In evaluating an adult patient whose 12SL-interpreted ECG lacks a prolonged QT diagnostic statement (assuming sinus rhythm <100 beats per minute and QRS duration <120 ms), physicians should examine the actual QTc value displayed on the report before concluding that this parameter is normal.
Epinephrine QT Stress Testing in the Evaluation of Congenital Long-QT Syndrome: Diagnostic Accuracy of the Paradoxical QT Response
TLDR
The epinephrine QT stress test can unmask concealed type 1 long-QT syndrome with a high level of accuracy and indicates inferior diagnostic utility in this setting.
An explainable algorithm for detecting drug-induced QT-prolongation at risk of torsades de pointes (TdP) regardless of heart rate and T-wave morphology
Genetic testing for risk stratification in hypertrophic cardiomyopathy and long QT syndrome: fact or fiction?
  • M. Ackerman
  • Medicine, Biology
    Current opinion in cardiology
  • 2005
TLDR
Genomic medicine has now entered the clinical practice as it pertains to the evaluation and management of both hypertrophic cardiomyopathy and long QT syndrome, with diagnostic utility of genetic testing for both diseases clearly evident.
Epinephrine-induced QT interval prolongation: a gene-specific paradoxical response in congenital long QT syndrome.
TLDR
Epinephrine provocation may help unmask some patients with concealed LQTS and strategically direct molecular genetic testing, and epinephrine-induced prolongation of the QT interval appears pathognomonic for LQT1.
Clinical Evaluation, Risk Stratification, and Management of Congenital Long QT Syndrome
Once considered an extremely rare and lethal arrhythmogenic peculiarity, the congenital long QT syndrome (LQTS) is understood today as a primary cardiac channelopathy that is far more common but less…
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