Long QT syndrome type 1 in an Australian indigenous patient

A 19-year-old Australian Indigenous woman presented after suffering a ventricular fibrillation cardiac arrest whilst swimming, leading to near drowning, in a remote Aboriginal community of South Australia. The patient was initially resuscitated by approximately 10 minutes of basic life support from a lifeguard, before receiving an automatic external defibrillation shock, and transferred to a regional intensive care unit where she received 24 hours of cooling. She retained normal cognitive function upon recovery. The patient had no prior history of palpitations, presyncope, or syncope. Inpatient ECG demonstrated a prolonged corrected QT interval (QTc) of 510 ms (Figure, A). QTc interval prolongation persisted during exercise and recovery. An echocardiogram and cardiac MRI demonstrated a structurally normal heart. A diagnosis of long QT syndrome was made, the patient was treated with a beta-blocker and an internal cardioverter defibrillator (ICD) was implanted. She has had no further clinical arrhythmias.