Abstract

Major advances in pediatric cardiology in recent decades, especially surgical techniques, have resulted in an increasing number of patients with congenital heart disease (CHD) surviving to adulthood. This has generated new challenges, particularly with regards to the late onset of complex arrhythmias. Abnormal anatomy, surgical scarring, chronic hypoxemia, hemodynamic compromise, neuro-hormonal abnormalities, and genetic factors can all contribute to creating a unique substrate for arrhythmia development. This review attempts to synthesize the current state of knowledge spanning the spectrum from underlying mechanisms of arrhythmias in patients with congenital heart disease to current ablative strategies. We discuss existing knowledge gaps and highlight important areas for future research.

Highlights

  • As a result of major advances achieved in the past decades in pediatric cardiology and surgical techniques regarding anatomical correction of congenital defects, most patients with congenital heart disease (CHD) reach adulthood, such that the population of survivors is increasing and aging [1]

  • Post-surgical scarring, and systemic factors contribute to establishing a unique substrate for arrhythmia development

  • We summarize the specific substrates, triggers, and modulators for arrhythmias in CHD

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Summary

Introduction

As a result of major advances achieved in the past decades in pediatric cardiology and surgical techniques regarding anatomical correction of congenital defects, most patients with congenital heart disease (CHD) reach adulthood, such that the population of survivors is increasing and aging [1]. This success is, tempered by the onset of late complications, including arrhythmias that are a major source of morbidity. AV, atrioventricular; CHD, congenital heart disease; ECG, electrocardiogram; VT, ventricular tachycardia

Congenital Heart Disease-Related Substrate
Post-Operative Substrate
Cardiovascular Risk-Related Substrate
Genetic Substrate
Abnormal Automaticity and Triggered Activity
Hemodynamic
Myocardial Ischemia
Neurohormonal Perturbations
Chronic Inflammation
Arrhythmia Diagnosis
Medical Therapy
Current Approaches and Outcomes
Technical Challenges to Reach Heart Chambers
Evaluation of Arrhythmias
Atrial Fibrillation
Conclusions
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