Sudden Cardiac Death in Dilated Cardiomyopathies

Abstract

Dilated cardiomyopathy (DCM) is the most prevalent form of cardiomyopathy, and sudden cardiac death (SCD) remains a common event in young patients. The disorder is highly heterogeneous and pediatric DCM often differs from adult DCM in etiologies, risk factors, and prognosis. Prognosis may be improving, likely secondary to specialized management. Both traditional and novel markers of risk have been studied in adults and are beginning to be applied to children with DCM. The major therapy for patients considered at risk remains implantable cardioverter-defibrillator (ICD) placement, although frequently this functions as a bridge to eventual transplantation.

Sudden cardiac death in the young: the bogeyman

“…However, aetiology can be determined only in 50% of the cases. 49,50 Despite a lack of agreement in the literature, we do not classify post-ischaemic ventricular dysfunction in the context of dilated cardiomyopathy.…”

“…The prevalence of dilated cardiomyopathy in the adult population is much higher. 50,51 Sudden cardiac death can occur in up to 10% of children with dilated cardiomyopathy, and it is usually caused by ventricular tachyarrhythmia secondary to left ventricular remodelling. Sudden cardiac death can also occur before the impairment of the ejection fraction.…”

Evaluating the Efficacy and Safety of Internal Cardioversion with Implantable Cardioverter Defibrillator Device for Atrial Fibrillation in Systolic Heart Failure Patients

“…ICD implantation rates are growing rapidly both for primary and secondary prevention of sudden cardiac death in patients with heart failure . Consequently, a significant proportion of patients with heart failure and ICD present with atrial arrhythmias.…”

The clinical management of relatives of young sudden unexplained death victims; implantable defibrillators are rarely indicated

“…While only one patient with DCM was commenced on the heart failure triad of β-blocker, ACE inhibitor and spironolactone all other DCM patients were already established on these medications prior to clinic attendance. Outside β-blockers, no other antiarrhythmic medication has been shown to prevent sudden death 23. No antiarrhythmic drug has been demonstrated to improve prognosis in ARVC although β-blockade is widely prescribed in view of the fact that a proportion of arrhythmic events in ARVC occur during exercise or isoprenaline infusion 24…”