Treatments for Long QT Syndrome LQTS in Children

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Contact the Inherited Cardiac Arrhythmia Program

Given the significance of family histo­ries, Dominic Abrams, MD, of the Cardi­ology Department at Boston Children's Hospital, has developed a new approach to treating inherited diseases—an approach that looks at the entire family rather than just the single patient. Clinicians at Boston Children’s, led by Abrams, and clinicians at Brigham & Women’s, led by Calem MacRae, created the Family Arrhythmia Clinic for this family-based approach. At this clinic, individuals and families gain easy and convenient access to a detailed evaluation, discussion and formulation of an integrated family plan. In a single, comprehensive visit, available every Friday, families are provided the convenience of a “one stop shop.” 

This all-day, all-inclusive approach was designed to make sure the whole family is looked after and is getting a clear, consistent message. It avoids the problem of having the family visit different centers, and allows us to identify similarities and variability between individuals in the family.

During the day-long meeting, chil­dren are typically seen in the morning and undergo specific investi­gations, while parents and other adult family members are seen in the afternoon at Brigham & Women’s Hospital. At the end of the day, findings are explained to the entire family, and a plan for further tests or treatment is presented. Not only does this strate­gy make clinical sense by allowing for more comprehensive interpretation of clinical and genetic data, but it also creates important research opportunities.

Occasionally, findings in one family shed new light on our understanding of the genetic basis of one specific condition and have sig­nificant implications for other patients,” Abrams explains.

This approach requires effective communication with families and individuals prior to their visit by our specialist Inherited Arrhythmia Program nurse, including obtaining:

  • a detailed summary of family relationships (i.e. pedigree)
  • copies of prior clinical and genetic reports
  • copies of family members autopsy reports (if relevant)

This information, along with any additional genetic results, will allow for the appropriate discussion in the clinic and minimize the need for repeat testing.

Clinical and genetic investigation of individuals with suspected inherited arrhythmic syndromes requires detailed evaluations by experienced physicians who have treated the spectrum of these patients—from the individual with no symptoms (but with subtle phenotypic or genetic findings), to the detailed cardiac and molecular autopsy following the sudden cardiac death of a related family member.

Most critical to producing the best outcomes is a comprehensive evaluation of all family members to establish phenotypic expression, assess segregation of genetic variants with the clinical disease process, and determine optimal treatment strategies.

Our plan will always be highly personalized, especially given the variability in all these conditions. Therefore, our risk stratification and management of arrhythmic conditions is highly individualized, determined by the specific genetic expression in any one individual. Yet we also encompass the individual’s plan within a clearly defined, overarching family strategy.

After the clinic visit, for those living at a distance from Boston Children’s, we will work with your local physicians to establish optimal, personalized care plans for all the members of your family.  Throughout, you will also have simultaneous access to the state-of-art specialist clinic at Boston Children’s.

In addition, clinicians at Boston Children’s will remotely follow up with these individuals through proprietary technology that uses online interaction, family-focused social media and other emerging technologies. 

Treatments for this condition may range from lifestyle changes to surgery.

  • Lifestyle modifications
    • Factors such as stress, caffeine, or alcohol can provoke arrhythmias. You may need to modify your diet.
    • Remaining adequately hydrated with water and/or sports drinks to maintain your electrolytes (sodium and potassium) before, during and after activities.
    • Your cardiologist may recommend restrictions during exercise or competitive sports.  
    • Medical Therapy

Beta-blocker or other medications may be prescribed to reduce the incidence of arrhythmias and risk for sudden cardiac arrest.

  • Ablation

This surgical procedure eliminates extra or abnormal electrical pathways within the heart that can cause fast or irregular heart rhythms.

  • Pacemaker

An electronic device that is surgically placed in the patient’s body and connected to the heart to regulate the heartbeat.

  • Implantable cardioverter-defibrillator (ICD)

A small device, similar to a pacemaker, is implanted under the skin, often in the shoulder area just under the collarbone. An ICD senses heart rate and, if it exceeds a programmed rate, delivers a small, electrical shock that slows the heart down. Newer ICDs are combined with a pacemaker and can deliver electrical signals to interrupt arrhythmias. ICDs are used for life-threatening fast arrhythmias such as ventricular tachycardia or ventricular fibrillation.

We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”
- Sandra L. Fenwick, President and CEO

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