However, the past 2 decades have witnessed the emergence of an expanded and effective risk stratification model, resulting in more reliable identification of those patients at increased SD risk, who may become candidates for the life-saving benefits of implantable cardioverter-defibrillators (ICDs), 1,9,11,13,[19][20][21][22][23][24][25][26][27] and advancements in heart transplantation, modern resuscitation techniques, and surgical management of outflow tract obstruction, as well. [9][10][11][12][13]23,23,[28][29][30][31] Whether these contemporary management options have resulted in reduced mortality rates for children and young adults with HCM is currently unresolved, and is the subject of the present clinical outcome investigation. Methods Patient SelectionDatabases from 2 major HCM tertiary referral centers, Minneapolis Heart Institute Foundation and Tufts Medical Center, were accessed and 474 consecutive patients <30 years of age were identified presenting between 1992 and 2013. Age at initial evaluation was 20.2±5.1 years: ≤10 years (n=10 patients), 11 to 15 years (n=94 patients); 16 to 20 years (n=173 patients); 21 to 25 years (n=117 patients); 26 to 29 years (n=80 patients). Age at diagnosis was 17±6 years with 276 patients (58%) <18 years of age.Background-Youthful age has been considered the time of greatest risk for patients with hypertrophic cardiomyopathy (HCM), largely because of the possibility of sudden death. The last 2 decades have witnessed more reliable identification of at-risk patients and utilization of implantable cardioverter-defibrillators for prevention of sudden death, and other contemporary treatment options. Whether such management advances have significantly altered the considerable mortality rate for young HCM patients remains unresolved. Methods and Results-We studied long-term outcome in 474 consecutive HCM patients between 7 and 29 years of age presenting at 2 referral institutions. Over 7.1±5.1 years of follow-up (6.0 [3.0, 10.0]), 452 patients (95%) survived, with 95% experiencing no or mild symptoms. HCM-related death occurred in 18 patients (3%; 0.54%/y): arrhythmic sudden death (n=12), progressive heart failure and heart transplant complications (n=5), or postoperatively (n=1). In contrast, aborted life-threatening events occurred in 63 other high-risk patients (13%) with implantable cardioverterdefibrillator interventions for ventricular tachyarrhythmias (n=31), resuscitated out-of-hospital cardiac arrest (n=20), or heart transplant for advanced heart failure (n=12), 1.8%/y, 3-fold higher than HCM mortality. Five-and 10-year survival (considering only HCM deaths) was high (97% and 94%, respectively), virtually identical to that reported in middle-aged adult HCM patients (98% and 94%, P=0.23). Conclusions-In a large hospital-based cohort of young HCM patients, representing an age group considered at greatest risk, low mortality rates can be achieved with the application of contemporary cardiovascular treatment strategies, largely because of reliable identification of high...

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AimThe purpose of this study was to determine if Optim™, a unique copolymer of silicone and polyurethane, protects Riata ST Optim and Durata implantable cardioverter-defibrillator (ICD) leads (SJM, St Jude Medical Inc., Sylmar, CA, USA) from abrasions that cause lead failure.Methods and resultsWe searched the US Food and Drug Administration's (FDA's) Manufacturers and User Device Experience (MAUDE) database on 13 April 2012 using the simple search terms ‘Riata ST Optim™ abrasion analysis’ and ‘Durata abrasion analysis’. Lead implant time was estimated by subtracting 3 months from the reported lead age. The MAUDE search returned 15 reports for Riata ST Optim™ and 37 reports for Durata leads, which were submitted by SJM based on its analyses of returned leads for clinical events that occurred between December 2007 and January 2012. Riata ST Optim™ leads had been implanted 29.1 ± 11.7 months. Eight of 15 leads had can abrasions and three abrasions were caused by friction with another device, most likely another lead. Four of these abrasions resulted in high-voltage failures and one death. One failure was caused by an internal insulation defect. Durata leads had been implanted 22.2 ± 10.6 months. Twelve Durata leads had can abrasions, and six leads had abrasions caused by friction with another device. Of these 18 can and other device abrasions, 13 (72%) had electrical abnormalities. Low impedances identified three internal insulation abrasions.ConclusionsRiata ST Optim™ and Durata ICD leads have failed due to insulation abrasions. Optim™ did not prevent these abrasions, which developed ≤4 years after implant. Studies are needed to determine the incidence of these failures and their clinical implications.