REVIEW URRENT C OPINION
Implantable cardioverter-defibrillators in congenital heart disease Blandine Monde´sert and Paul Khairy
Purpose of review Sudden cardiac death is a leading cause of mortality in patients with congenital heart disease (CHD), such that implantable cardioverter-defibrillators (ICDs) are a critical component of care. Objectives of this review are to highlight recent advances regarding ICDs in CHD, with a focus on clinical indications, technical issues and solutions, and patient outcomes. Recent finding Evolving ICD indications in CHD are primarily derived from observational research or extrapolations from the general adult literature. Technical challenges to device implantation include obstructed vasculature or conduits, intracardiac shunts with their attendant risk for systemic thromboemboli, and lack of venous access to the heart. In selected patients, tailored epicardial systems may be considered that include subcutaneous, retrocardiac, and/or venous (e.g., azygous) coils. Alternatively, an entirely subcutaneous ICD may be a reasonable option in patients with no bradycardia or antitachycardia pacing indications. Long-term complications include inappropriate shocks, lead failure, reduction in quality of life, shock-related anxiety, and impaired sexual function. Summary Although ICDs undeniably save lives, challenges to applying this technology to patients with CHD include the paucity of evidence-based data to guide patient selection, technical challenges related to venous access, patient size, anatomic complexities, and a high rate of complications. Keywords congenital heart disease, implantable cardioverter-defibrillators, sudden cardiac death
INTRODUCTION Declining mortality rates in developed countries are giving rise to a rapidly growing population of survivors born with heart defects [1]. Among the varied and multiorgan complications encountered by the aging heterogeneous cohort of patients with congenital heart disease (CHD), arrhythmias are a major source of morbidity and the primary drivers of hospitalizations and healthcare resource utilization [2–5]. Moreover, heart failure and sudden cardiac death (SCD) of presumed arrhythmic etiology are the leading causes of mortality, inciting much interest in implantable cardioverter-defibrillators (ICDs). As indications expand and technologies improve, an increasing number of children and young adults with CHD are receiving ICDs [6]. Although there is little doubt that ICDs save lives, the downside of therapy, including a high rate of lead-related complications and inappropriate shocks, must be balanced against the probability of experiencing a potentially lethal tachyarrhythmia [7–9].
The objectives of this review are to highlight recent advances in ICDs pertinent to CHD, with a focus on clinical indications, implantation and extraction issues, and patient outcomes.
INDICATIONS Patients with CHD represent a small minority (
Implantable cardioverter-defibrillators in congenital heart disease Blandine Monde´sert and Paul Khairy
Purpose of review Sudden cardiac death is a leading cause of mortality in patients with congenital heart disease (CHD), such that implantable cardioverter-defibrillators (ICDs) are a critical component of care. Objectives of this review are to highlight recent advances regarding ICDs in CHD, with a focus on clinical indications, technical issues and solutions, and patient outcomes. Recent finding Evolving ICD indications in CHD are primarily derived from observational research or extrapolations from the general adult literature. Technical challenges to device implantation include obstructed vasculature or conduits, intracardiac shunts with their attendant risk for systemic thromboemboli, and lack of venous access to the heart. In selected patients, tailored epicardial systems may be considered that include subcutaneous, retrocardiac, and/or venous (e.g., azygous) coils. Alternatively, an entirely subcutaneous ICD may be a reasonable option in patients with no bradycardia or antitachycardia pacing indications. Long-term complications include inappropriate shocks, lead failure, reduction in quality of life, shock-related anxiety, and impaired sexual function. Summary Although ICDs undeniably save lives, challenges to applying this technology to patients with CHD include the paucity of evidence-based data to guide patient selection, technical challenges related to venous access, patient size, anatomic complexities, and a high rate of complications. Keywords congenital heart disease, implantable cardioverter-defibrillators, sudden cardiac death
INTRODUCTION Declining mortality rates in developed countries are giving rise to a rapidly growing population of survivors born with heart defects [1]. Among the varied and multiorgan complications encountered by the aging heterogeneous cohort of patients with congenital heart disease (CHD), arrhythmias are a major source of morbidity and the primary drivers of hospitalizations and healthcare resource utilization [2–5]. Moreover, heart failure and sudden cardiac death (SCD) of presumed arrhythmic etiology are the leading causes of mortality, inciting much interest in implantable cardioverter-defibrillators (ICDs). As indications expand and technologies improve, an increasing number of children and young adults with CHD are receiving ICDs [6]. Although there is little doubt that ICDs save lives, the downside of therapy, including a high rate of lead-related complications and inappropriate shocks, must be balanced against the probability of experiencing a potentially lethal tachyarrhythmia [7–9].
The objectives of this review are to highlight recent advances in ICDs pertinent to CHD, with a focus on clinical indications, implantation and extraction issues, and patient outcomes.
INDICATIONS Patients with CHD represent a small minority (
