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13th March 2024
Clinical features
Congenital LQTS can be split syndromes based on the phenotype:
| Inheritance and genetics | Phenotype | |
| Romano-Ward syndrome | Autosomal dominant
Any genetic mutation causing LQTS |
Pure cardiac phenotype – prolonged QT and cardiac events
Not associated with deafness |
| Jervell and Lange-Nielsen syndrome | Autosomal recessive
KCNQ1 |
Severe LQTS and sensorineural deafness
Cardiac events in infancy |
| Andersen-Tawil syndrome | Autosomal dominant | Periodic paralysis
Ventricular tachyarrhythmias |
Typically, LQTS presents by the time someone is 30 years old. Presentation may be with syncope or syncope followed by seizures. Symptoms, including chest pain, breathlessness or palpitations, will usually precede syncopal episodes. There may be distinct triggers (see above), such as swimming or being startled. Sudden cardiac arrest is an uncommon but possible presentation of LQTS. All of these cardiac events are the result of arrhythmias caused by a prolonged QT interval.
Some people with congenital LQTS are asymptomatic and remain so throughout their lives. Diagnosis may occur through screening of an affected family member.
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