Congenital long-QT syndrome is caused by a gene mutation. The mutation causes delayed repolarisation of the heart, reflected in a prolonged QT interval on an ECG. A long-QT interval is associated with an increased risk of sudden cardiac death. Long-QT syndrome is one of the causes of sudden cardiac death in young athletes. The risk of death with long-QT is increased by activities such as swimming, vigorous exercise, loud noises, and sudden frights, although most people with long-QT who die suddenly die during their sleep. People with long-QT syndrome are often asymptomatic. My fit healthy brother died suddenly and unexpectedly in bed one night at the age of 33 years, leaving a wife and three small children. We have subsequently discovered that we have one of the gene mutations that causes long-QT syndrome running in our family. This video (10 minutes) presents the story of a patient in my practice who has congenital long-QT syndrome, told in her own words. It tells the story of her road to diagnosis, what it is like to live with symptomatic long-QT and the risk of sudden death, and the effect that the diagnosis and treatment have had on her life. The video also presents information about congenital long-QT and an interview with a cardiologist who specialises in the diagnosis and management of inherited cardiac diseases.
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