AIMS: Clinical presentation, occurrence of sudden infant death, and results of the available therapies in the largest group of patients with short QT syndrome (SQTS), studied so far, are reported. METHODS AND RESULTS: Clinical history, physical examination, electrocardiogram (ECG), exercise stress testing, electrophysiological study, morphological evaluation, genetic analysis and therapy results in 29 patients with SQTS and personal and/or familial history of cardiac arrest are reported. The median age at diagnosis was 30 years (range 4-80). In all subjects, structural heart disease was excluded. Eighteen patients were symptomatic (62%): 10 had cardiac arrest (34%) and in 8 (28%) this was the first clinical presentation. Cardiac arrest had occurred in the first months of life in two patients. Seven patients had syncope (24%); 9 (31%) had palpitations with atrial fibrillation documented even in young subjects. At ECG, patients exhibited a QT interval < or = 320 ms and QTc < or = 340 ms. Fourteen patients received an implantable cardioverter-defibrillator (ICD) and 10 hydroquinidine prophylaxis. At a median follow-up of 23 months (range 9-49), one patient received an appropriate shock from the ICD; no patient on hydroquinidine had sudden death or syncope. CONCLUSION: 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.
AIMS: Clinical presentation, occurrence of sudden infant death, and results of the available therapies in the largest group of patients with short QT syndrome (SQTS), studied so far, are reported. METHODS AND RESULTS: Clinical history, physical examination, electrocardiogram (ECG), exercise stress testing, electrophysiological study, morphological evaluation, genetic analysis and therapy results in 29 patients with SQTS and personal and/or familial history of cardiac arrest are reported. The median age at diagnosis was 30 years (range 4-80). In all subjects, structural heart disease was excluded. Eighteen patients were symptomatic (62%): 10 had cardiac arrest (34%) and in 8 (28%) this was the first clinical presentation. Cardiac arrest had occurred in the first months of life in two patients. Seven patients had syncope (24%); 9 (31%) had palpitations with atrial fibrillation documented even in young subjects. At ECG, patients exhibited a QT interval < or = 320 ms and QTc < or = 340 ms. Fourteen patients received an implantable cardioverter-defibrillator (ICD) and 10 hydroquinidine prophylaxis. At a median follow-up of 23 months (range 9-49), one patient received an appropriate shock from the ICD; no patient on hydroquinidine had sudden death or syncope. CONCLUSION: 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.
Authors: Peter A Noseworthy; Aki S Havulinna; Kimmo Porthan; Annukka M Lahtinen; Antti Jula; Pekka J Karhunen; Markus Perola; Lasse Oikarinen; Kimmo K Kontula; Veikko Salomaa; Christopher Newton-Cheh Journal: Circ Cardiovasc Genet Date: 2011-04-21
Authors: J R Moffett; R A Price; S M Anderson; M L Sipos; A V Moran; F C Tortella; J R Dave Journal: Cell Mol Life Sci Date: 2003-10 Impact factor: 9.261
Authors: Charles Antzelevitch; Guido D Pollevick; Jonathan M Cordeiro; Oscar Casis; Michael C Sanguinetti; Yoshiyasu Aizawa; Alejandra Guerchicoff; Ryan Pfeiffer; Antonio Oliva; Bernd Wollnik; Philip Gelber; Elias P Bonaros; Elena Burashnikov; Yuesheng Wu; John D Sargent; Stefan Schickel; Ralf Oberheiden; Atul Bhatia; Li-Fern Hsu; Michel Haïssaguerre; Rainer Schimpf; Martin Borggrefe; Christian Wolpert Journal: Circulation Date: 2007-01-15 Impact factor: 29.690
Authors: Nitin Mathur; Subeena Sood; Sufen Wang; Ralph J van Oort; Satyam Sarma; Na Li; Darlene G Skapura; J Henri Bayle; Miguel Valderrábano; Xander H T Wehrens Journal: Circ Arrhythm Electrophysiol Date: 2009-12