- Prolonged ventricular re-polarisation = prolongation of the QT interval
- Risk of Torsade de pointes and sudden death
- Congenital or acquired
- Romano-Ward syndrome - autosomal dominant.
- Lange-Nielsen syndrome - autosomal recessive (assoc congenital deafness.
- F > M, usually childhood or adolescence.
- Once identified, first degree relatives should be screened.
|Males or females <15 years old||
|Males > 15 years||
|Females > 15 years||
- Palpitations, syncope/near syncope, seizures, cardiac arrest.
- QTc = QT/R-R-2. >0.45 sec abnormal
- Abnormal T-wave (notched or biphasic)
- T-wave alternans
- "lifestyle modifications," (avoidance competitive sports and of all drugs known to prolong QT interval) (list).
- Treat with β-blockers (shorten the QT interval, reduce risk of Torsade and sudden death).
- High risk patients - implantable cardioverter-defibrillators (ICDs)
- Left cervicothoracic sympathectomy (block sympathetic to heart so reduce event rate).