Implantable Cardioverter-Defibrillator Therapy in Pediatric and Congenital Heart Disease

Implantable cardioverter-defibrillators (ICDs) have become a mainstay in preventing sudden cardiac death in children and individuals with congenital heart disease. Decisions around device implantation must balance long-term benefits against risks of complications, including lead failure, infection and inappropriate shocks. Anatomical variations and ongoing somatic growth pose unique challenges for lead placement, device selection and generator sizing. Emerging technologies—such as subcutaneous and extravascular systems—are designed to reduce intravascular lead-related morbidity while providing effective defibrillation. Optimal patient selection requires stratification of arrhythmic risk according to underlying pathology, whether channelopathy, cardiomyopathy or post-surgical residual defects. Device programming algorithms and follow-up strategies have been refined to minimise inappropriate therapies without compromising sensitivity to life-threatening arrhythmias. Long-term data indicate that, although paediatric patients experience higher complication rates than adults, careful multidisciplinary management and personalised programming can yield favourable outcomes. Globally, the expansion of specialist centres and telemonitoring has improved access to advanced ICD therapy and enhanced surveillance of device performance in varied healthcare settings.

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Implantable Cardioverter-Defibrillator Therapy in Pediatric and Congenital Heart Disease publication trend

The graph below shows the total number of articles in implantable cardioverter-defibrillator therapy in pediatric and congenital heart disease across all publications each year (not limited to Nature Index journals).

Technical terms

Primary prevention: Implantation of an ICD in patients at high risk of life-threatening arrhythmia who have not yet experienced one.

Secondary prevention: Implantation of an ICD following a documented episode of ventricular tachycardia, fibrillation or cardiac arrest.

Subcutaneous ICD (S-ICD): A defibrillator system placed entirely beneath the skin, avoiding intravascular leads.

Extravascular ICD (EV-ICD): A device with leads positioned outside the vascular system, often in a substernal or epicardial location.

Inappropriate shocks: Unnecessary or unwarranted defibrillation therapy delivered in response to non-ventricular arrhythmias or artefacts.

References

1. Implantable cardioverter defibrillator therapy in paediatric patients for primary vs. secondary prevention. EP Europace (2024).
2. Minimally Invasive Implantation of an Extravascular Implantable Cardioverter-Defibrillator Device in a 2 Year Old. Annals of Thoracic Surgery Short Reports (2024).
3. A cluster of inappropriate shocks in a pediatric S-ICD patient - how to troubleshoot?. Indian Pacing and Electrophysiology Journal (2023).