Implantable cardioverter-defibrillator therapy in patients with congenital long-QT syndrome: A long-term follow-up

ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities

“…384 -386 ICD implantation is recommended for selected patients with recurrent syncope despite drug therapy, sustained ventricular arrhythmias, or sudden cardiac arrest. 349,351,352,387,388 Furthermore, use of the ICD for primary prevention of SCD may be considered when there is a strong family history of SCD or when compliance or intolerance to drugs is a concern. 349,351,352,387,388 The clinical manifestations of a long-QT mutation may be influenced by the specific gene involved and the functional consequences of the mutation in that gene.…”

Longevity of Sprint Fidelis Implantable Cardioverter-Defibrillator Leads and Risk Factors for Failure

“…Long-term studies have shown that lead complications have been relatively high in patients who receive ICD therapy for Brugada 16 and congenital long-QT syndromes. 17 Precisely how these clinical variables affect the Fidelis pace-sense conductors is unknown. However, the pattern of Fidelis conductor fractures is consistent with a nonrandom time-dependent mechanical failure mode, and it is likely that young active adults with these inherited cardiac diseases mechanically stress Fidelis leads more vigorously than older sedentary patients who have severe ischemic cardiomyopathy and heart failure.…”

ICD Implantations in the Pediatric and Young Adult Population

“…Although the ICD implant frequency is greatest among LQT3 patients, the greatest save rate has occurred among LQT2 women who were assessed to be at high risk (Horner et al, 2010). However, when used in a high-risk LQTS population, ICD therapy seems to be a safe option (Monnig et al, 2005). Risk factors for appropriate ICD shocks are longer QTc intervals and being survivors of a cardiac arrest.…”

“…Risk factors for appropriate ICD shocks are longer QTc intervals and being survivors of a cardiac arrest. However, betablockers should always be added to ICD therapy, while some patients might benefit from additional antibradycardia pacing, prolonged detection time, and a rate-smoothing algorithm to prevent recurrent episodes (Monnig et al, 2005). …”