long Q-T syndrome
long Q-T syndromeA condition that is most common in otherwise healthy young women, which is evoked by physical exercise (in particular swimming) or mental activity (e.g., severe fright or anger, stress), resulting in episodic syncope or, if stimulus is extreme, sudden death related to increased autonomic tone (e.g., while exercising on a hot day) accompanied by sudden onset of ventricular arrhythmia. Once diagnosed, all blood relatives should have an EKG since a prolonged Q-T interval due to delayed repolarisation increases the risk of re-entry phenomenon and malignant ventricular arrhythmia (e.g.,”torsades de points”).
Long Q-T syndrome may be induced by drugs (e.g., antiarrhythmics, phenothiazine, tricyclic antidepressants and lithium), metabolic and electrolyte imbalances, low-energy diets, CNS and autonomic nervous system disease, coronary artery disease, and mitral valve prolapse. Prolonged Q-T interval is also a symptom in Jervell-Lange-Nielsen syndrome, an autosomal recessive [MIM 220400] condition accompanied by deafness and Romano-Ward syndrome, an autosomal-dominant [MIM 192500] condition without deafness.
Syncope, aborted cardiac arrest or sudden death.
Often have a strong family history of sudden cardiac death at young age.
Long Q-T interval, right bundle branch block and elevated ST segment.
Correct hypomagnesaemia and hypopotassaemia (hypokalaemia); beta blockers (propranolol, nadolol); implantable cardioverter-defibrillator; stellectomy.