Short QT Syndrome
Short QT Syndrome (SQTS) is a very rare heart disease. Most of the public has probably never heard about it and there are still a great number of physicians who have only limited knowledge about it - Preben Bjerregaard MD
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How To Diagnose Short QT Syndrome
The cornerstone in the diagnosis is a short QT interval, which, unfortunately, is difficult to define, and the QT interval can be difficult to measure and is not the same all the time. Before the discovery of Short QT Syndrome there was no universal agreement as to when a QT interval was short, and even though such an agreement still does not exist, approximate figures for the lower limit of a QT interval at a heart rate of 60 beats per minute are 360 milliseconds for men and 370 milliseconds for women.
Resources
Short QT Syndrome
This Website was established to spread the word about a heart disease called Short QT Syndrome which is easily overlooked and unfortunately life-threatening.
Congenital Short QT Syndrome (CSQTS)
Currently, the use of implantable cardioverter-defibrillators (ICD) is the most accepted treatment option for individuals with CSQTS.
CV clinic – Short QT Syndrome
The QTc is a calculated QT interval which is standardised to a heart rate of 60 beats per minute and this is the number that should be reviewed when looking at an ECG. SQTS diagnosis is based on the evaluation of symptoms, family history and 12-lead ECG. Features such as syncope and palpitations should be asked about, along with family history of syncope, sudden death or AF at a young age.
Diagnosis And Management Of Short QT Syndrome Patients
Establishing a definition of Short QT Syndrome including symptomatology and QT interval duration is still a work in progress. It is clear, however, that SQTS is a rare, life-threatening, inherited heart disease presenting as SCD or aborted SCD in 34% and a family history of SCD in 15%.
Recent Advances in Short QT Syndrome
Short QT syndrome is a highly malignant inherited cardiac disease characterized by ventricular tachyarrhythmias leading to syncope and sudden cardiac death. It is responsible of lethal episodes in young people, mainly infants.
Short QT syndrome
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.
The Long and Short of It: Insights Into the Short QT Syndrome
The SQTS is a genetically heterogeneous disorder with mutations described in 3 genes that encode for potassium channels (KCNH2, KCNQ1, KCNJ2).
How To Diagnose Short QT Syndrome
QTc intervals calculated from QT intervals at heart rates below 60 beats per minute are often falsely shorter than the actual QT interval at 60 beats per minute, which can lead to over-diagnosing of Short QT in people with a slow heart rate as seen for instance in younger male persons, who often have a slow pulse. If a person is under suspicion of having a Short QT Syndrome it is important to make several ECG recordings, often a 24-hour ambulatory ECG recording or recordings during physical activity can be helpful.
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