Key result
Multimodal risk stratification tools, including AI-ECG and 3D CMR, enhance sudden cardiac death risk estimation in congenital heart disease but require further validation for shockable rhythms.
Why the study?
Sudden cardiac death remains a feared and difficult-to-predict outcome in patients with congenital heart disease.
SCD risk prediction in CHD is evolving toward a multimodal, individualized approach, though new models and technologies do not eliminate the uncertainty inherent in predicting rare events.
Should not yet change SCD risk stratification in CHD; leaves open multimodal tool validation for shockable rhythms.
PURPOSE OF REVIEW: Sudden cardiac death (SCD) remains a feared and difficult-to-predict outcome in patients with congenital heart disease (CHD). This review examines the latest evidence in risk stratification, with a focus on limitations of existing models and the mechanistic and statistical complexities that hinder individualized decision-making. RECENT FINDINGS: New multivariable risk scores for repaired tetralogy of Fallot and systemic right ventricle have improved prognostic resolution. Artificial intelligence-enabled ECG algorithms have shown promise in early identification of high-risk individuals with repaired tetralogy of Fallot. In parallel, three-dimensional cardiac magnetic resonance imaging has been leveraged to delineate arrhythmogenic isthmuses, enhancing substrate-guided interventions. While these tools enhance risk estimation, they require validation specific to the prediction of shockable terminal rhythms, improved interpretability, and integration into individualized decision frameworks. SUMMARY: SCD risk prediction in CHD is evolving toward a multimodal, individualized approach that emphasizes probabilistic reasoning, shared decision-making, and epistemic humility. Although new models and technologies offer incremental gains, they do not eliminate the uncertainty inherent in predicting rare events. The application of population-based tools to individual patients must be interpreted cautiously, recognizing that SCD represents a final common pathway for diverse pathophysiological processes, and that decisions about ICD implantation entail complex trade-offs.
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Khairy et al. (2025) conducted a review in Congenital heart disease. Risk stratification tools (multivariable scores, AI-ECG, 3D CMR) was evaluated on Sudden cardiac death risk prediction. Multimodal risk stratification tools, including AI-ECG and 3D CMR, enhance sudden cardiac death risk estimation in congenital heart disease but require further validation for shockable rhythms.
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