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Based on our evaluation and review of the available literature, we believe that an AED, is warranted and medically necessary for young people with SADS conditions. The HAT (Home Automatic External Defibrillator Trial) study is not applicable to young people with conditions that carry a high risk of life-threatening arrhythmias, such as long QT syndrome. Other studies have shown that bystander use of AEDs "significantly improves survival". People with diagnosed SADS conditions have a known increase in risk for sudden cardiac arrest and, also, respond well to defibrillation.
Importantly, the CMS (Centers for Medicare and Medicaid Systems) issued a policy effective 1/1/2004, to provide Medicare coverage for AEDS prescribed to patients with "familial or inherited conditions with a high risk of life-threatening ventricular tachyarrhythmias such as Long QT and hypertrophic cardiomyopathy". Long QT Syndrome is judged by CMS to be a significant risk in and of itself without any other qualifying conditions.
Long QT Syndrome (LQTS) is the most common cardiac channelopathy, affecting approximately 1/3,000 individuals in the United States with 500-1,000 new carriers born each year. This syndrome causes cardiac arrhythmias in seemingly healthy and often young individuals and can lead to syncope, seizures, cardiac arrest, and sudden death. LQTS is one of the more common causes of sudden death in young people, resulting in 2,000 to 3,000 deaths per year.1 In fact, because LQTS is unassociated with anatomic cardiac markers identifiable during life or at autopsy, its impact as a cause of premature death is probably underestimated.2 While there is no cure, proper medical management including combinations of lifestyle modification, β-blocker therapy, AEDs and (ICDs) can reduce the risk of untoward events that lead to death.
"Cardiopulmonary resuscitation (CPR) combined with bystander use of an automated external defibrillator (AED) more than doubled the chances of surviving out-of-hospital cardiac arrest compared with using CPR alone. Researchers from the Resuscitations Outcomes Consortium (ROC), a network of 11 urban and rural communities in the United States and Canada involved in studies of prehospital emergency care, analyzed data gathered over one year."
1The Sudden Arrhythmia Death Syndromes (SADS) Foundation: Long QT Syndrome Fact Sheet, July 2002. http://www.sads/About-SADS-Conditions/Long-QT-Syndrome
2 Maron BJ, et al. Impact of laboratory molecular diagnosis on contemporary diagnostic criteria for genetically transmitted cardiovascular diseases: hypertrophic cardiomyopathy, Long-QT syndrome, and Marfan syndrome. A statement for healthcare professionals from the Councils on Clinical Cardiology, Cardiovascular Disease in the Young, and Basic Science, American Heart Association]. Circulation 1998;98:1460-1471.
[1] ROC Investigator: Myron L. Weisfeldt, M.D., Johns Hopkins University. Released by the American Heart Association, November, 5, 2007
[i] ROC Investigator: Myron L. Weisfeldt, M.D., Johns Hopkins University. Released by the American Heart Association, November, 5, 2007