Congenital long QT syndrome: considerations for primary care physicians.

@article{Levine2008CongenitalLQ,
  title={Congenital long QT syndrome: considerations for primary care physicians.},
  author={Ethan Levine and S. Rosero and A. Budzikowski and A. Moss and W. Zareba and J. Daubert},
  journal={Cleveland Clinic journal of medicine},
  year={2008},
  volume={75 8},
  pages={
          591-600
        }
}
Congenital long QT syndrome is an inherited disorder of cardiac repolarization that predisposes to syncope and to sudden death from polymorphic ventricular tachycardia. The disorder should be suspected when the electrocardiogram shows characteristic QT abnormalities, or when there is a family history of long QT syndrome or of an event that raises suspicion of long QT syndrome, such as sudden death, syncope, or ill-defined "seizure" disorder. We can now classify some types of congenital long QT… Expand
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References

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Genetics of acquired long QT syndrome.
TLDR
The molecular and cellular mechanisms underlying this acquired or drug-induced form of long QT syndrome are summarized, approaches to the analysis of a role for DNA variants in the mediation of individual susceptibility are described, and it is proposed that these concepts may be generalizable to common acquired arrhythmias. Expand
Diagnostic Criteria for the Long QT Syndrome An Update
TLDR
The availability of effective therapy for this often lethal disease emphasizes the importance of early and accurate diagnosis, and there is frequently a delay in the diagnosis of LQTS, and patients with syncope are often misdiagnosed. Expand
The long QT syndrome family of cardiac ion channelopathies: A HuGE review*
TLDR
While phenotypic ascertainment remains a mainstay in the clinical setting, SSCP and DHPLC-aided DNA sequencing are a standard part of mutational investigation, and direct sequencing on a limited basis is now commercially available for patient diagnosis. Expand
Clues or miscues? How to make the right interpretation and correctly diagnose long-QT syndrome.
The congenital long-QT syndrome (LQTS) is an inherited condition associated with a prolongation of the QT interval on the ECG characterized by syncope, seizures, and sudden death secondary toExpand
Possible Bradycardic Mode of Death and Successful Pacemaker Treatment in a Large Family with Features of Long QT Syndrome Type 3 and Brugada Syndrome
TLDR
The clinical features and efficacy of pacemaker therapy in preventing sudden death in this family with features of both long QT syndrome type 3 and the Brugada syndrome are detailed. Expand
Diagnostic Miscues in Congenital Long-QT Syndrome
TLDR
Determinants for discordance included overestimation of QTc, diagnosing LQTS on the basis of “borderline”QTc values, and interpretation of a vasovagal fainting episode as an LQ TS-precipitated cardiac event. Expand
ECG T-wave patterns in genetically distinct forms of the hereditary long QT syndrome.
TLDR
Three separate genetic loci for the long QT syndrome including mutations in two cardiac ionic channel genes were associated with different phenotypic T-wave patterns on the ECG, providing insight into the influence of genetic factors on ECG manifestations of ventricular repolarization. Expand
Role of implantable cardioverter defibrillator therapy in patients with long QT syndrome.
Long QT syndrome (LQTS) is one of several primary electrical disorders or hereditary arrhythmia syndromes along with the short QT syndrome, Brugada syndrome, and catecholaminergic polymorphicExpand
Identification of a common genetic substrate underlying postpartum cardiac events in congenital long QT syndrome.
TLDR
There is a relatively gene-specific molecular basis underlying cardiac events during the postpartum period in LQTS, and this association between post partum cardiac events and LQT2 can facilitate strategic genotyping. Expand
Drug Challenge with Epinephrine or Isoproterenol for Diagnosing a Long QT Syndrome: Should We Try This at Home?
  • S. Viskin
  • Medicine
  • Journal of cardiovascular electrophysiology
  • 2005
TLDR
Since symptoms in the common types of LQTS are commonly triggered by stress, it is only logical to use sympathetic stimulation, in the form of adrenaline or isoproterenol infusion, as a challenge test to unravel a LQ TS in questionable cases. Expand
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