The short QT syndrome is a recently described genetic arrhythmogenic disorder, characterized by abnormally short QT intervals and a high incidence of sudden death and atrial fibrillation. Clinical manifestations may also be present in infants; a family history of cardiac sudden death is often present. Gain-of-function mutations in 3 genes encoding potassium channels and loss-of-function mutations in 2 genes encoding the cardiac L-type calcium channel have been identified. Today, the first choice therapy is implantable cardioverter-defibrillator implantation; however, pharmacologic treatment with hydroquinidine, which prolongs QT and reduces the inducibility of ventricular arrhythmias, may be proposed for children and probably for elderly asymptomatic patients
Short QT syndromes
Mazzanti, AndreaMembro del Collaboration Group
2010-01-01
Abstract
The short QT syndrome is a recently described genetic arrhythmogenic disorder, characterized by abnormally short QT intervals and a high incidence of sudden death and atrial fibrillation. Clinical manifestations may also be present in infants; a family history of cardiac sudden death is often present. Gain-of-function mutations in 3 genes encoding potassium channels and loss-of-function mutations in 2 genes encoding the cardiac L-type calcium channel have been identified. Today, the first choice therapy is implantable cardioverter-defibrillator implantation; however, pharmacologic treatment with hydroquinidine, which prolongs QT and reduces the inducibility of ventricular arrhythmias, may be proposed for children and probably for elderly asymptomatic patientsI documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
