Short QT syndrome historical perspective: Difference between revisions
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Revision as of 17:05, 3 September 2012
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
The syndrome was first described by Dr. Prebe Bjerregaard MD, DMSc in 1999[1][2].
Description of the First Patient with Short QT Syndrome
Shalon Hill, a 17 year old white female, underwent laparoscopic cholecystectomy at Anderson Hospital, Maryville, Illinois in 1999 which was complicated by atrial fibrillation with a rapid ventricular response (RVR) at 150-200 beats/min along with acute pulmonary edema[1][3]. The atrial fbrillation with RVR was treated with DC cardioversion and she was discharged to home in normal sinus rhythm on digoxin. The atrial fibrillation recurred 6 weeks later and she was found at that time to have a short QT interval of 225 mseconds which was treated with prophylactic therapy with propafenone. She then remained asymptomatic for 6 months and the propafenone was discontinued. However, the atrial fibrillation recurred 2 months after the propafenone was discontinued, and it was therefore resumed. She remained asymptomatic on propafenone, but an AICD was implanted given reports from around the world of sudden cardiac death.
Description of the First Family with Short QT Syndrome
EKGs of the first patient's family members were analyzed. The QT interval of her 21 year old brother was 240 msec, the QT interval of her 84 year old maternal grandfather was 240 msec, and the QT interval of her 51 year old mother was 230 msec. The EKG of here father was normal[1][4].
He brother was asymptomatic, and on August 13, 2003 was found to have inducible ventricular fibrillation on programmed electrical stimulation. This was treated with implantation of an implantable cardioverter defibrillator. Subsequently he complained of occasional palpitations and paroxysmal atrial fibrillation with a rapid ventricular response was noted on interrogation of the ICD.
Her mother is a 51 year old healthy white female with a history of 3 episodes of sustained palpitations and paroxysmal atrial fibrillation. She has remained asymptomatic on propafenone since April, 2003. Programmed electrical stimulation on September 29, 2003 induced both atrial and ventricular fibrillation and an AICD was implanted.
Her maternal grandfather was an 84 year old white male who had chronic atrial fibrillation, coronary artery disease and hypertension who died following an embolic stroke.
References
- ↑ 1.0 1.1 1.2 Gussak I, Brugada P, Brugada J, Wright RS, Kopecky SL, Chaitman BR, Bjerregaard P (2000). "Idiopathic short QT interval: a new clinical syndrome?". Cardiology. 94 (2): 99–102. doi:47299 Check
|doi=value (help). PMID 11173780. Retrieved 2012-09-03. - ↑ http://www.shortqtsyndrome.org/short_qt_history.htm
- ↑ http://www.shortqtsyndrome.org/short_qt_history.htm
- ↑ http://www.shortqtsyndrome.org/short_qt_history.htm