Title:Current Indications for Implantable Cardioverter Defibrillators in Non-Ischemic Cardiomyopathies and Channelopathies
Volume: 10
Issue: 2
Author(s): Esteban Gonzalez-Torrecilla, Angel Arenal, Felipe Atienza, Tomas Datino, Loreto Bravo, Pablo Ruiz, Pablo Avila and Francisco Fernandez-Aviles
Affiliation:
Keywords:
Guidelines, implantable cardioverter defibrillator, cardiomyopathies, channelopathies,
primary prevention, risk score, sudden cardiac death.
Abstract: Current indications for implantable cardioverter defibrillators (ICDs) in patients with channelopathies
and cardiomyopathies of non-ischemic origin are mainly based on non-randomized evidence. In patients
with nonischemic dilated cardiomyopathy (NIDCM), there is a tendency towards a beneficial effect on
total mortality of ICD therapy in patients with significant left ventricular (LV) dysfunction. Although an important
reduction in sudden cardiac death (SCD) seems to be clearly demonstrated in these patients, a net
beneficial effect on total mortality is unclear mostly in cases with good functional status. Risk stratification
has been changing over the last two decades in patients with hypertrophic cardiomyopathy (HCM). Its risk
profile has been delineated in parallel with the beneficial effect of ICD in high risk patients. Observational results
based on “appropriate” ICD interventions do support its usefulness both in primary and secondary SCD
prevention in these patients. Novel risk models quantify the rate of sudden cardiac death in these patients on
individual basis. Less clear risk stratification is available for cases of arrhythmogenic right ventricular cardiomyopathy
(ARVC) and in other uncommon familiar cardiomyopathies. Main features of risk stratification
vary among the different channelopathies (long QT syndrome -LQTS-, Brugada syndrome, etc) with great
debate on the management of asymptomatic patients. For most familiar cardiomyopathies, ICD therapy is the
only accepted strategy in the prevention of SCD. So far, genetic testing has a limited role in risk evaluation
and management of the individual patient. This review aims to summarize these criticisms and to refine the
current indications of ICD implantation in patients with cardiomyopathies and major channelopathies.
