The long QT syndrome (LQTS) is a heart condition that slows the recovery of heart cells (repolarization) following each contraction. This abnormality is manifest on the ECG as prolongation of the QT interval. Having the condition makes the heart prone to fast and dangerous heart rhythms that can lead to fainting or worse sudden death. Such dangerously fast heart rhythms associated with the long QT syndrome are often described as ventricular arrhythmia, ventricular fibrillation, or Torsade de Pointes (TdP).
The long QT syndrome can be due to inherited abnormalities in channels in heart cells that regulate the flow of electrically-charged molecules into and out of the heart cells. However, sometimes the condition is exacerbated or caused by drugs. At Johns Hopkins Hospital, patients with the long QT syndrome are typically assessed by cardiologists and genetic counselors, thus optimizing the treatment of affected patients and identification of potentially affected relatives.
Genetic Counselors who treat this condition:
Nicole M. Johnson, ScM, CGC