Cardiology in review
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The inherited long QT syndrome is caused by mutations of at least 5 ion channel genes. Mutations of the cardiac sodium ion channel gene and 3 potassium channel genes have been identified to this time. A genetic locus on chromosome 4 has been identified, but no gene has been discovered as of yet. ⋯ The principal treatment is beta-blocker therapy. Appropriate dosing, with ascertainment of efficacy and compliance with administration, are the key elements in therapeutic success. Molecular physiology-based strategies are being considered, including the use of sodium channel blockers in LQT3 and potassium administration in LQT1 patients.