The following are key points to remember from this state-of-the-art review of heart failure with arrhythmia in adults with congenital heart disease:

1. One third of adults with congenital heart disease (ACHD) will develop decompensated systemic or pulmonary venous congestion over the course of their adult life, and more than half will develop arrhythmias.
2. Sudden cardiac death (SCD) is an important contributor to mortality in the ACHD population. While there are limited data to inform the indications for primary prevention implantable cardioverter-defibrillator (ICD) therapy in ACHD patients with left ventricular ejection fraction (LVEF) ≤35%, expert consensus statements support ICD therapy for such patients with biventricular physiology and New York Heart Association (NYHA) functional class II or III despite optimal medical therapy.
3. In adults with tetralogy of Fallot (TOF), pulmonary valve replacement improves symptoms and may halt progression of right ventricular (RV) dilatation. It does not generally have a significant positive impact on arrhythmia burden and risk. The presence of concomitant LV systolic and diastolic dysfunction identifies those at the highest risk of short-term mortality due to heart failure and SCD.
4. Primary prevention ICD is a class IIA recommendation in TOF patients with multiple risk factors for SCD.
5. Patients with systemic right ventricles face increased risk for supraventricular arrhythmias, ventricular arrhythmias, and conduction disturbances. Beta-blockers have been shown to independently reduce the risk for appropriate ICD shocks for patients with D-transposition of the great arteries (TGA) after atrial switch procedures.
6. The role for cardiac resynchronization therapy (CRT) in the ACHD population continues to evolve. There may be a role for their use in arteries (congenitally corrected TGA [CCTGA]). The most recent European Society of Cardiology ACHD guidelines suggest that biventricular pacing be considered in all CCTGA patients with anticipated >40% ventricular pacing.
7. Patients with systemic right ventricles also have an increased risk for SCD. Primary prevention ICDs may be considered in patients with advanced systemic RV dysfunction (EF <35%) in the presence of additional risk factors such as severe tricuspid regurgitation, prolonged QRS duration, and atrial tachyarrhythmias.
8. At experienced centers, excellent outcomes can be achieved with catheter ablation for patients with Fontan circulation and supraventricular arrhythmias. For patients with refractory arrhythmias, Fontan conversion may be considered.
9. Multisite pacing for Fontan patients may have a role, although there is not sufficient evidence for its use at this time. It is reasonable to consider CRT for Fontan patients with a high burden of ventricular pacing and wide QRS.

Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Prevention, Vascular Medicine, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Interventions, CHD and Pediatrics and Quality Improvement, Acute Heart Failure, Interventions and Structural Heart Disease, Interventions and Vascular Medicine

Keywords: Arrhythmias, Cardiac, Arterial Switch Operation, Cardiac Resynchronization Therapy, Catheter Ablation, Death, Sudden, Cardiac, Defibrillators, Implantable, Dilatation, Fontan Procedure, Heart Defects, Congenital, Heart Failure, Hyperemia, Primary Prevention, Risk Factors, Stroke Volume, Tachycardia, Tetralogy of Fallot, Transposition of Great Vessels, Tricuspid Valve Insufficiency, Ventricular Function, Left

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