Long QT syndrome (LQTS) is a genetic condition that effects how the heart “recharges” (repolarization) after each beat. Long QT can lead to a life threatening arrhythmia called Torsades de Pointe.
As an international referral center for children with complex congenital heart disease, the University of Michigan C.S. Mott Children's Hospital Congenital Heart Center is one of the largest and best pediatric heart programs in the United States. University of Michigan pediatric heart specialists offer state-of-the-art treatment and management of congenital heart conditions, including a comprehensive range of support services and long-term follow up care for children and their families. Our team has deep expertise caring for children with heart rhythm disorders, from diagnosis and treatment through life-long follow up care.
Symptoms of long QT syndrome
Symptoms include fainting (syncope), seizures-like episodes, or sudden death. Many patients are asymptomatic and are diagnosed through a routine ECG or due to a family history of sudden death or fainting with activity.
Diagnosing long QT syndrome
The diagnosis of long QT syndrome is based primarily on the finding of a prolonged QT interval on ECG. Family history, symptoms and genetic testing also play a key role in diagnosis and disease risk.
Treatment of long QT syndrome
Treatment includes a medication called a beta blocker which helps to decrease the likelihood of a fatal arrhythmia and fainting. This medication is prescribed by a cardiologist/electrophysiologist and must be taken every day. In some instances, an implantable cardioverter defibrillator (ICD) is necessary for patients who have a high risk of sudden death or have previously had an episode of fatal arrhythmia.
Life with long QT syndrome
Patients with long QT syndrome need life-long follow up with a cardiologist/electrophysiologist. The risk of sudden death is variable at different points in a patient’s life and ongoing treatment is needed. Your cardiology/electrophysiology team may restrict certain physical activities.
Certain medications can prolong the QT interval and should be avoided in patients with long QT syndrome. Be sure to check with your pharmacist or physician before taking any additional medications. Family members of patients with long QT syndrome may also carry the disease and should be evaluated by a cardiologist.
Why choose C.S. Mott Children’s Hospital for long QT syndrome care
The electrophysiology program at C.S. Mott Children’s Hospital is the Midwest’s largest EP program. Our team offers evaluation, diagnosis, genetic testing, treatment and consultation for patients with suspected or diagnosed long QT syndrome.
U-M’s Congenital Heart Center is uniquely positioned to care for heart rhythm disorder patients from infancy through adulthood, as a children’s hospital directly attached to an adjacent top-ranked adult cardiovascular center. Our pediatric specialists work in coordination with U-M adult cardiologists who have expertise in long QT syndrome to provide seamless care for the family.