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Short QT syndrome.

ABSTRACT: The QT interval on an electrocardiogram signifies the time required for the heart to repolarize after depolarization. It has long been appreciated that a long QT interval predisposes patients to life-threatening ventricular arrhythmia. Short QT syndrome is a newly described disease characterized by a shortened QT interval and by episodes of syncope, paroxysmal atrial fibrillation or life-threatening cardiac arrhythmias. The syndrome usually affects young and healthy people with no structural heart disease and may be present in sporadic cases as well as in families. Our understanding of a new disease has rarely benefitted so quickly from research in genetics, molecular biology and biophysics. It was first described in 2000 in a handful of patients, and since then 3 different genes associated with the disease and the biophysical basis have been described, and therapy has been made available. Here we review the current understanding of the pathophysiology, clinical presentation and treatment of short QT syndrome.

SUBMITTER: Brugada R

PROVIDER: S-EPMC1283503 | biostudies-literature | 2005 Nov

REPOSITORIES: biostudies-literature

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Short QT syndrome.

Brugada Ramon R Hong Kui K Cordeiro Jonathan M JM Dumaine Robert R

CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne 20051101 11

The QT interval on an electrocardiogram signifies the time required for the heart to repolarize after depolarization. It has long been appreciated that a long QT interval predisposes patients to life-threatening ventricular arrhythmia. Short QT syndrome is a newly described disease characterized by a shortened QT interval and by episodes of syncope, paroxysmal atrial fibrillation or life-threatening cardiac arrhythmias. The syndrome usually affects young and healthy people with no structural hea ...[more]

PMID: 16301704

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Project description:BackgroundShort QT syndrome (SQTS) is a rare syndrome and affects different types of genes. However, data on differences of clinical profile and outcome of different SQTS types are sparse.MethodsWe conducted a pooled analysis of 110 SQTS patients. Patients have been diagnosed between 2000 and 2017 at our institution (n = 12) and revealed using a literature review (n = 98). 29 studies were identified by analysing systematic data bases (PubMed, Web of Science, Cochrane Libary, Cinahl).Results67 patients with genotype positive SQTS origin and 43 patients with genotype negative origin were found. A significant difference is documented between the sex with a higher predominance of male in genotype negative SQTS patients and predominance of females in genotype positive SQTS patients (male 52% versus 84%, female 45% versus 14%; p = 0.0016). No relevant difference of their median age (genotype positive 27 ± 19 versus genotype negative 29 ± 15; p = 0.48) was found. Asymptomatic patients and patients reporting symptoms such as syncope, sudden cardiac death, atrial flutter and ventricular fibrillation documented in both groups were similar except atrial fibrillation (genotype positive 19% versus genotype negative 0%; p = 0.0055). The QTc interval was not significantly different in both groups (genotype positive 315 ± 32 versus genotype negative 320 ± 19; p = 0.30). The treatments (medical treatment and ICD implantation) in both groups were comparable. Electrophysiology studies were not significantly higher documented in patients with genotype positive and negative origin (24% versus 9%; p = 0.075). Events at follow up such as VT, VF, and SCD were not higher presented in patients with genotype positive (13% versus 9%) (p = 0.25). 54% of genotype positive SQTS patients showed SQTS 1 followed by STQS 2 (21%) and SQTS 3 (10%).ConclusionsThe long-term risk of a malignant arrhythmic event is not higher in patients with genotype positive. However, patients with genotype positive present themselves more often with AF with a female predominance. Also, other events at follow up such as syncope, atrial flutter and palpitation were not significantly higher (9% versus 0%; p = 0.079).

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Short QT syndrome.

Publications

Short QT syndrome.

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