Long QT Syndrome: A Case Report, Genomics, and Clinical Implications

  Long QT syndrome (LQTS) is a hereditary disorder in which the majority of affected individuals present with QT prolongation on electrocardiograms (ECGs), arising from delayed ventricular repolarization. This commonly arrhythmia-associated genetic syndrome can lead to sudden cardiac death (SCD) and increased propensity for arrythmogenic syncope. The authors describe the case of a young patient who presented with episodes of syncope, prolonged QTc interval on ECG, and family history of SCD. He subsequently received an ICD for protection against SCD based on his prior clinical history. The article discusses the clinical manifestations, electrocardiographic (ECG) findings, management of LQTS, and role of the clinician in testing, teaching, and counseling the affected patients and families.