Dear Readers,

Prevention of stroke is one of the cornerstones of therapy for patients with atrial fibrillation (AF). The most effective drug to prevent thromboembolism in patients with AF and risk factors for stroke is warfarin (Coumadin). The ACTIVE-W trial showed us that a combination of clopidogrel (Plavix) and aspirin cannot substitute for warfarin. However, as most health care providers and patients are well aware, there are major limitations to the use of warfarin — a significant risk of bleeding, a narrow therapeutic window, frequent food and drug interactions, and a need for frequent blood draws to guide dosing. Fortunately, there are numerous drugs under development that might replace warfarin, including direct thrombin and factor Xa inhibitors, and oral forms of heparin. Until then, our only drug option is warfarin. Furthermore, even if a substitute for warfarin becomes available, we will still have to contend with bleeding complications.

Some form of congenital heart disease is present in 75/1,000 live births. ¹ The majority of these patients live into adulthood, with approximately one half million adults living with congenital heart disease in the United States. Sudden cardiac death is now the leading cause of mortality in this patient population.^2,3

Advances in surgery for congenital heart disease have saved or prolonged the lives of thousands. As with any successful operation or procedure, there are trade-offs for success. Many years removed from their initial surgery, these patients are now at risk for morbidity and mortality from bradyarrhythmias and tachyarrhythmias.

In this article, author Dr. Kathy Glatter discusses the use of genetic testing for finding cardiac syndromes such as long QT syndrome.

Case History

A 34-year-old female with long QT syndrome (LQTS) was referred to our institution for evaluation. Starting at age 19, she had experienced four documented cardiac arrests, all of when she was several months postpartum with each of her four children. Her cardiac catheterization and echocardiogram results were normal. However, an electrocardiogram (ECG) showed a prolonged QTC (corrected QT interval) of 480 ms. Her mother was healthy and had a QTC on ECG of 460 ms, but the patient’s maternal aunt had died suddenly at five months postpartum. A normal QTC interval for a female is 450 or less and 440 or less for a male.