Short QT syndrome: a case report and review of literature.

Abstract

The short QT syndrome has been recently recognised as a genetic ion channel dysfunction. This new clinical entity is associated with an incidence of sudden cardiac death, syncope, and atrial fibrillation in otherwise healthy individuals. The distinctive ECG pattern consists of an abnormally short QT interval, a short or even absent ST segment and narrow T waves. A 30-year-old resuscitated woman with short QT syndrome is described together with an example of the classic ECG characteristics. A short-coupled variant of torsade de pointes was reveal on Holter recordings. The implantable cardioveter defibrillator seems to be the therapy of choice to prevent from sudden cardiac death. Quinidine proved to be efficient in prolonging the QT interval and rendering ventricular tachyarrhythmias non-inducible in patients with a mutation in KCNH2 (HERG). Our preliminary data suggest amiodarone combined with beta-blocker may be helpful in treating episodes of polymorphic ventricular tachycardia for patients with an unknown genotype. Because the short QT syndrome often involves young patients with an apparently normal heart, it is imperative for physicians to recognize the clinical features of the short QT syndrome in making a timely correct diagnosis.

• Authors: Li Xiong Lu, Wei Zhou, Xingyu Zhang, Qin Cao, Kanglong Yu, Changqing Zhu
• Journal: Resuscitation (Resuscitation) Vol. 71 Issue 1 Pg. 115-21 (Oct 2006) ISSN: 0300-9572 [Print] Ireland
• PMID: 16942825 (Publication Type: Case Reports, Journal Article, Review)

Chemical References

• Adrenergic beta-Antagonists
• ERG1 Potassium Channel
• Ether-A-Go-Go Potassium Channels
• KCNH2 protein, human
• Amiodarone

Topics

• Adrenergic beta-Antagonists (administration & dosage)
• Adult
• Amiodarone (administration & dosage)
• Arrhythmias, Cardiac (drug therapy, physiopathology)
• ERG1 Potassium Channel
• Electrocardiography
• Ether-A-Go-Go Potassium Channels (genetics)
• Female
• Heart Conduction System (physiopathology)
• Humans
• Mutation
• Syndrome
• Torsades de Pointes (physiopathology)
• Ventricular Fibrillation (physiopathology)