Diagnosis and management of short QT syndrome.

@article{Bjerregaard2018DiagnosisAM,
  title={Diagnosis and management of short QT syndrome.},
  author={Preben Bjerregaard},
  journal={Heart rhythm},
  year={2018},
  volume={15 8},
  pages={
          1261-1267
        }
}

Rare but lethal short QT syndrome: most recent understanding of the disease

This rare and under-studied condition is described, steps to diagnosis and treatment modalities are highlighted, particularly in South America where there is a lack of studies and understanding of this disease.

[Long and short QT syndromes : Emergency treatment and secondary prophylaxis].

Primary prevention is based on betablocker therapy and implantation of an implantable cardioverter-defibrillator (ICD), if appropriate, if appropriate and ICDs are the only reliable protection against SCD.

Short QT Syndrome: A Comprehensive Genetic Interpretation and Clinical Translation of Rare Variants

An exhaustive study of variants currently catalogued as deleterious in short QT syndrome determined that only nine have a conclusive pathogenic role; all definitively pathogenic variants are located in KCNQ1, KCNH2, or KCNJ2.

Long-Term Prognosis of Short QT Interval in Asian Patients: Multicenter Retrospective Cohort Study

In this Asian population, SQTI was associated with AF and VA/SCA and early repolarization, tall T wave, and U wave were significantly more frequent in patients with SQTI than the patients without SQTI.

Long-term prognosis of short QT interval in Korean patients: a multicenter retrospective cohort study

In this subset of Korean population, SQTI was associated with AF and VA/SCA, and early repolarization, tall T wave, and U wave were significantly more frequent in patients with SQTI than in those without.

Molecular genetic markers of QT interval duration and sudden cardiac death: literature review

The aim of this review was to study and analyze the available literature data on the relationship of molecular genetic markers with the duration of the QT interval and identify a large amount of genetic data that may be a promising approach to quantifying the risk of SCD.

Clinical Genetics of Inherited Arrhythmogenic Disease in the Pediatric Population

An update on the state of the art in the genetic diagnosis of inherited arrhythmogenic disease in the pediatric population is provided, focusing on emerging publications on gene curation for genotype–phenotype associations, cases of genetic overlap and advances in the classification of variants of uncertain significance.

Impact of Antiarrhythmic Drugs on the Outcome of Short QT Syndrome

Hydroquinidine (HQ) increases the corrected QT interval and prevents VAs in the majority of the patients in this cohort and is safe for use in SQTS patients, particularly due to its low rate of side effects.

Clinical and Genetic Characteristics of Congenital Long QT Syndrome

Clinical and genetic characteristics of different types of congenital long QT syndrome are discussed and effective therapy requires molecular genetic diagnosis to accurately determine the type of this pathology.

References

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The short QT syndrome: proposed diagnostic criteria.

Short QT syndrome: clinical findings and diagnostic-therapeutic implications.

SQTS carries a high risk of sudden death and may be a cause of death in early infancy; ICD is the first choice therapy; hydroquinidine may be proposed in children and in the patients who refuse the implant.

Short QT syndrome in a boy diagnosed on screening for heart disease

During treadmill exercise stress test, QT and QU intervals responded differently to heart rate changes, suggesting a mechanoelectrical hypothesis for the origin of the U wave.

Long-term follow-up of patients with short QT syndrome.

Short QT Syndrome: A Familial Cause of Sudden Death

The short QT syndrome is characterized by familial sudden death, short refractory periods, and inducible ventricular fibrillation, which is related to a high risk of sudden death in young, otherwise healthy subjects.

Short QT syndrome in a 14-year-old patient: The first pediatric case from Turkey

This paper presents a 14-year-old male patient whose elder brother and father had died because of SD and who presented to us with the complaint of syncope, who was identified to have a short QT interval (SQTI) in his electrocardiogram result.

Novel Insight Into the Natural History of Short QT Syndrome

Short QT Syndrome Manifesting with Neonatal Atrial Fibrillation and Bradycardia

A subset of children who present with early-onset AF and a slow ventricular response rate who were found to have the short QT syndrome (SQTS) are described, which may present in utero as persistent bradycardia with postnatal ECG showing a very shortQT interval.
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