Diagnosis At present, there are no diagnostic criteria for SQTS. [litfl.com]

Clinical presentation The clinical presentation of SQTS is also diverse with a high penetrance but a great variability of expression between different families and even among members of the same family. SQTS is characterized by a high lethality. [escardio.org]

All probands presented with a QTc below 320 ms without evident structural heart disease. [ncbi.nlm.nih.gov]

Short Q-T syndrome is a congenital defect, which means it is present at birth. The condition is inherited from at least one parent. [my.clevelandclinic.org]

Self-terminating idiopathic ventricular fibrillation presenting as syncope: a 40-year follow-up report. J Intern Med. 1990;227:211-3. Campbell TJ. Characteristics of cardiac action potentials in marsupials. J Comp Physiol. 1989;158:759-62. [medigraphic.com]

Short QT syndrome: pharmacological treatment. J Am Coll Cardiol. 2004;43:14949. Schimpf R, Antzelevitch C, Hsu LF, Schickel S, Pollevick GD, Cordeiro JM, et al. [medigraphic.com]

In asymptomatic patients with a short QTc interval, observation without treatment is recommended 3. [e-cardiogram.com]

Treatment Options At present, the only effective treatment is implantation of an ICD. The main problem with this is T-wave oversensing and inappropriate shocks due to the tall, narrow T waves seen in SQTS. [litfl.com]

Even though ICD is the only effective treatment, it also has specific problems in these patients. [escardio.org]

Gaita F, Giustetto C, Bianchi F, Schimpf R, Haissaguerre M, Calo L et al (2004) Short QT syndrome: pharmacological treatment. J Am Coll Cardiol 43:1494–1499 PubMed CrossRef 12. [springermedizin.de]

However, it should be emphasised that the prognosis of patients with asymptomatic SQTS still remains undefined. [ncbi.nlm.nih.gov]

Interestingly, four of these five genes have been implicated in the etiology of long QT syndrome but with mutations in the opposite sense, that’s to say, loss of function mutations instead of gain of function mutations of potassium channels. [escardio.org]

[…] appropriate discharges Prevention of symptomatic episodes of atrial fibrillation However, drugs must be used with caution since the long-term efficacy of drug therapy in preventing serious arrhythmic events has been studied only in SQTS 1 patients and [escardio.org]

But, it is important for anyone who knows they have SQTS to see a doctor for regular care to prevent sudden cardiac death or cardiac arrest. What causes SQTS? Short Q-T syndrome is a congenital defect, which means it is present at birth. [my.clevelandclinic.org]

This information is important because it supports the need to consider implanting an ICD for secondary prevention of CA even in young SQTS patients. Anttonen O, Junttila J, Giustetto C, et al. T-Wave morphology in short QT syndrome. [e-cardiogram.com]

Reprogramming the decay delay, sensitivity or both generally prevents inappropriate discharges. [ncbi.nlm.nih.gov]

Short QT syndrome: successful prevention of sudden cardiac death in an adolescent by implantable cardioverter-defibrillator treatment for primary prophylaxis. Heart Rhythm. 2005;2:4167. Patel Ch, Yan GX, Antzelevitch C. [medigraphic.com]