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Smaller patients in general weigh less than approximately 15 kg, and larger patients weigh more than approximately 15 kg. þ The precise definition of "medical therapy that is either not effective or associated with intolerable adverse effects" is left up to the practitioner and family to decide. In general, however, the threshold for ineffectiveness and intolerability should be higher in smaller patients. For example, failure or
Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease, with an annual risk of sudden cardiac death (SCD) estimated at 1 %. Limited data are available regarding both the risk of SCD in the young HCM population and the use of implantable cardioverter-defibrillators (ICDs). This retrospective study included all patients with HCM who underwent ICD implantation for primary or secondary prevention of SCD before the age of 30 years at five institutions between 1995 and 2009. There were 99 devices implanted in 73 patients. Appropriate shocks occurred for 11 % of all the patients. None of the previously identified conventional risk factors for SCD in HCM patients were associated with increased risk of appropriate shocks in the young study cohort. During a median follow-up period of 2.4 years, inappropriate shocks occurred for 22 % of the patients. Older age at implant was associated with a decreased risk of inappropriate shock. Those who underwent implantation in the earlier decade had a higher incidence of inappropriate shocks. Late complications including lead fracture or dislodgement, generator malfunction, and infection occurred for 32 % of the patients. Three patients died (4 %), one of whom had an arrhythmic sudden death. A greater proportion of primary prevention implantations was performed for patients from the latter decade. Over time, ICD use in young HCM patients has become increasingly primary prevention oriented. Shock rates mirror those reported in adult series, and there is a substantial incidence of device complications.
Implantable cardioverter defibrillators (ICDs) are being used with increasing frequency in children and young adults. Our aim was to examine the appropriateness and frequency of ICD discharges in children and young adults, to compare the effectiveness of ICDs when placed for primary or secondary prevention, and to provide time-dependent analysis of ICD discharges. Data were collected from seven institutions on 210 patients <30 years of age who underwent ICD implantation from October 1992 to January 2007. Median age at implant was 15.4 years with a follow-up average of 3.3 years. Heart disease was categorized as electrical (n = 90, 42%), cardiomyopathic (n = 62, 30%), or congenital heart disease (n = 58, 28%). ICDs are increasingly placed for primary prevention. There are increased appropriate ICD discharges for ICDs placed for secondary prevention (52%) versus primary prevention (14%) at 5 years. There is no difference in the risk of inappropriate discharges between primary and secondary prevention indications. There is an increased risk for inappropriate therapy in the congenital heart disease population. An increasing number of ICDs are being placed for primary prevention in young patients, a marked shift in practice during the last two decades. The benefits of ICDs remain greater in secondary than in primary-prevention patients. In both groups, approximately 25% of patients received inappropriate discharges within 5 years of implant. Patients with congenital heart disease are the most affected by inappropriate discharges.
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