What is the life expectancy of someone with long QT syndrome?

Individuals with long QT syndrome (LQTS) can live a normal lifespan with great quality of life, particularly if they are identified prior to their first cardiac event and have worked closely with healthcare providers to develop a detailed monitoring, prevention, and treatment plan. However, depending on the type of LQTS and the gene involved, the risk for life threatening cardiac events is higher than in the general population, particularly from preteen year to the 20s. In about 10-15% of undiagnosed individuals living with LQTS, sudden cardiac death is the first sign of the disease. In diagnosed individuals living with treated LQTS and their relatives (who may or may not be treated), the cumulative mortality is 6-8% for LQTS type 1, 2, and 3. Different types of LQTS can be broken out of that estimate by type. For example, one study found that the cumulative mortality rates were:
2% in children ages 0-18 and 5% in adults aged 19-40 years in people living with LQTS type 1
3% in children ages 0-18 and 7% in adults aged 19-40 years in people living with LQTS type 2
7% in children ages 0-18 and 5% in adults aged 19-40 years in people living with LQTS type 3
In individuals living with LQTS over 40 years, cardiac events are rare and often associated with low potassium levels (hypokalemia) or taking medications that make the QT interval longer or cause hypokalemia. Having said this, individuals with type 3 LQTS do have an increased risk for cardiac events through adulthood.
The best chance of increasing life expectancy in an individual with LQTS is to avoid medications, substances, and events that lengthen the QT interval (link to that list) and work closely with a heart doctor who is experienced with LQTS and able to advise, monitor, and treat the condition with the most up-to-date information. For many individuals this may include taking medications such as a beta blocker or sodium channel blocker, possibly surgery to place an implantable cardioverter-defibrillator (ICD) and/or left cardiac sympathetic denervation (LCSD). It is also important to have regular appointments checking on the effectiveness of the existing treatment plan and watching for other health issues, particularly during childhood when children may need frequent changes in medication dose as they grow. Having automatic external defibrillators available at home, at school, and activities or sports restrictions may be recommended by a doctor for some individuals with LQTS.

Other Questions About Long QT syndrome

• Will my newborn be tested for long QT syndrome?
• Where is there a center of excellence in long QT syndrome?
• Where can I find clinical trials on long QT syndrome?
• When do people present with symptoms of long QT syndrome?
• What tests are required for a diagnosis of long QT syndrome?