@entity12396 : a familial cause of XXXX .

@entity1164 @entity1 @entity944 @entity1163 @entity12396 @entity2224 @entity299 @entity1167
BACKGROUND: A prolonged QT interval is associated with a risk for life-threatening events. However, little is known about prognostic implications of the reverse-a short QT interval. Several members of 2 different families were referred for @entity1163 , palpitations, and resuscitated @entity1167 in the presence of a positive family history for @entity1164 . Autopsy did not reveal any structural @entity299 . All @entity1 had a constantly and uniformly short QT interval at ECG. METHODS AND RESULTS: Six @entity1 from both families were submitted to extensive noninvasive and invasive work-up, including serial resting ECGs, echocardiogram, cardiac MRI, exercise testing, Holter ECG, and signal-averaged ECG. Four of 6 @entity1 underwent electrophysiological evaluation including programmed ventricular stimulation. In all subjects, a structural @entity299 was excluded. At baseline ECG, all @entity1 exhibited a QT interval <or=280 ms (QTc <or=300 ms). During electrophysiological study, short atrial and ventricular refractory periods were documented in all and increased ventricular vulnerability to fibrillation in 3 of 4 @entity1 . CONCLUSIONS: The @entity12396 is characterized by familial @entity944 , short refractory periods, and inducible @entity2224 . It is important to recognize this ECG pattern because it is related to a high risk of @entity944 in young, otherwise healthy subjects.
