LQTS: Long QT Syndrome. Inherited Heart Disease Clinic

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1 2011 LQTS: Long QT Syndrome Inherited Heart Disease Clinic

2 What is LQTS? LQTS is a rare heart rhythm disorder. It causes a disruption of the electrical signal that stimulates your heart to beat. It occurs in about 1:10,000 people. In a normal heartbeat, an electrical signal travels through the heart cells until it reaches the heart muscle and causes it to beat (or contract). In order for the electrical signal to flow properly, it must jump from cell to cell. For this to happen, each cell has special doors (or channels) that open and close quickly to allow the signal to enter. In LQTS these doors do not work properly. So, the electrical signal that was sent to trigger a heartbeat may not pass through the door properly. This may lead to a deadly heart rhythm. What causes LQTS? LQTS is mainly an inherited disorder (genes passed from one generation to the next). Members of families who are affected with this disease have a 50:50 chance of inheriting the genetic defect.

3 It is caused by an abnormality in one the genes that produce the channel s (or doors that control the entry of potassium or sodium into the heart cells). If your LQTS is hereditary, your doctor may recommend genetic counselling for you and your family. Certain medications may also cause the disorder. Symptoms Families and individuals identified with LQTS present in different ways. Some report the sudden death of a family member at a young age, have a history of fainting spells, seizures, palpitations, chest pain and/or blackout spells. If you or any of your family members have any of these symptoms, they should be reported to your doctor for further testing. Diagnosis The 2 main tests that help to determine if you have LQTS are: Electrocardiogram (ECG): An electrical tracing of your heartbeat is used to measure different parts of the electrical tracing picture.

4 Exercise Stress Test (EST): You will be asked to walk on a treadmill while your blood pressure, heart rate and heartbeat are recorded. Other tests may also be needed or suggested by your cardiologist. They will be discussed with you at that time, if needed. Genetic testing: This may be offered/ available to some people with this kind of heart rhythm problem. Your doctor or genetic counsellor will discuss this with you. Treatment Family inherited causes of LQTS are not curable, but some treatments may make you feel better. Your treatment will depend on your medical and family history and how you are feeling. Your doctor will review all of your test reports. You and your doctor will decide on the best treatment for you. Some of these may be: a medication called a beta-blocker, a pacemaker (to help control heart rate) and sometimes an internal defibrillator (ICD to detect and treat dangerous heart rhythms). It is also important to be aware of medications that may make the condition worse and should be avoided.

5 Activity and LQTS: How much can I do? If you are diagnosed with LQTS, your doctor will discuss what activities are safe for you and what activities you should avoid. These GENERAL GUIDELINES may vary from person to person: AVOID strenuous exercise as this can often trigger blackouts. DO NOT compete in competitive sports. Competitors tend to push themselves past their physical limitation. NEVER swim alone. Swimming can bring on a blackout event and therefore, may lead to drowning. BE AWARE that sudden events may trigger a potentially deadly rhythm (a sudden arousal or a startling noise when sleeping from a ringing of an alarm clock or telephone). It is recommended that these items not be used or volumes turned off/down when sleeping or napping. KNOW the medications that can make the problem worse. Some of these are over the counter medications. An updated list can be found at: Make sure your family and friends are aware of your condition and symptoms and that they know what to do if you have any of your symptoms.

6 For More Information Visit pub/guide/disease/electric/ longqtsyndrome.htm Clinic Staff Dr. Martin Gardner FRCP(C), FACC Dr. Chris Gray MD, FRCP(C) Victoria Newman-Burd RN, BScN, CCRP Dr. Sarah Dyack, MD, FRCPC, FCCMG Amy Crowley MS-CGC Julie Hatheway MS-CGC Looking for more information on this topic? Contact your local public library for books, videos, magazine articles and online health information. For a list of public libraries in Nova Scotia go to Capital Health promotes a smoke-free and scent-free environment. Please do not wear perfumed products. Thank You! Capital Health, Nova Scotia Funding for the Inherited Heart Disease Clinic provided by Medtronic of Canada Prepared by: Inherited Heart Disease Clinic, QEII, Halifax Designed and Printed by: CH Audio Visual and Printing Departments WG Revised Oct The information in this pamphlet is to be updated every 3 years.