Evaluation and treatment of pediatric patients with congenital or acquired long QT interval syndromes.
The long QT syndrome should be considered when evaluating patients with syncope. Prolongation of the QT interval and abnormalities of T wave morphology due to abnormal ventricular repolarization characterize the syndrome. In the past decade, molecular genetics has revealed that abnormal repolarization is the result of gene mutations encoding integral ion channels that generate the cardiac action potential. Eight subgroups of long QT syndrome associated with five genes have been described to date. The explosion in research in this area has led to a greater understanding of the clinical expression of autosomal dominant (Romano-Ward syndrome), autosomal recessive (Jervell and Lange-Nielsen syndrome) and acquired forms of the disease. This has also led to investigation in the area of genotype-specific therapy. The purpose of this review is to outline the strides made in the field of molecular genetics and update the reader on the recent advances in diagnosis and treatment of the long QT syndrome.
Phillips
J R.
JR
Division of Pediatric Cardiology, Cook Children's Heart Center, Fort Worth, TX, USA
Case
C L.
CL