Short QT syndrome

Finally, the ICH E14 guideline does not address the possible consequences of QT/ QTc shortening, which may be associated with significant arrhythmias [5,167]. Digitalis intoxication is a known example of drug-induced short QT syndrome associated with polymorphic ventricular tachycardia [ 168]. It should be acknowledged that this is an area of active research and no guidelines can be put forward at the moment. 

Wolpert, C., Schimpf, R., Veltmann, C., Giustetto, C., Gaita, F. and Borggrefe, M. (2005) Clinical characteristics and treatment of short QT syndrome. Expert Review of Cardiovascular Therapy, 3, 611-617. 

GF, gain of function LF, loss of function LQT, long QT syndrome PCCD, progressive cardiac conduction disorder SQT, short QT syndrome. 

Section 3.5 reviews a different set of channelopathies comprising cases of short QT syndrome (SQTS). While these channelopathies and their drug-induced phenotypic counterparts are not of central importance in this book s discussions, they are certainly worthy of inclusion in this chapter. 

The short QT syndrome is characterized by familial sudden death, short refractory periods, and indncible ventricnlar fibrillation. It is important to recognize this ECG pattern becanse it is related to a high risk of sudden death in young, otherwise healthy subjects. 

Comparisons of the incidence of the congenital syndromes show that short QT syndrome is much rarer than long QT syndrome, similar to the findings of short QT intervals compared with long QT intervals in the general population. 

Antzelevitch C, Francis J (2004) Congenital short QT syndrome. Indian Pacing Electrophysiol J 4 46-49... 

Brugada R, Hong K, Dumaine R et al (2004) Sudden death associated with short QT syndrome linked to Mutations in HERG. Circulation 109 30-35... 

Gaita F, Giustetto C, Bianchi F et al (2003) Short QT Syndrome a familial cause of sudden death. Circulation 108 965-970... 

Rudic B, Schimpf R, Borggrefe M (2014) Short QT syndrome review of diagnosis and treatment. Arrhythm Electrophysiol Rev 3 76-79... 

Borchert B, Lawrenz T, Stellbrink C (2006) Long and short QT syndrome. Herzschrittmacherther Elektrophysiol 17 205-210... 

Priori SG, Pandit SV, Rivolta I, Berenfeld O, Ronchetti E, Dhamoon A, NapoUtano C, Anumonwo J, di Barletta MR, Gudapakkam S, Bosi G, Stramba-Badiale M, Jalife J (2005) A novel form of short QT syndrome (SQT3) is caused by a mutation in the KCNJ2 gene. Circ Res 96 800-807... 

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