Key result
Strain and CMR fibrosis markers improve arrhythmia risk prediction beyond RV volumes in repaired Fallot.
Why the study?
Anatomical repair of tetralogy of Fallot does not eliminate long-term cardiovascular risk, as chronic pulmonary regurgitation imposes volume overload on the right ventricle and triggers dynamic remodeling and arrhythmogenic substrate formation. This review synthesizes the pathophysiological mechanisms, imaging assessment methods, and prognostic implications of right ventricular remodeling in repaired tetralogy of Fallot.
Does multimodal imaging integrating myocardial strain and fibrosis markers improve prognostic risk stratification for ventricular arrhythmias and sudden cardiac death compared to isolated volumetric parameters in patients with repaired Tetralogy of Fallot?
Does multimodal imaging integrating myocardial strain and fibrosis markers improve prognostic risk stratification for ventricular arrhythmias and sudden cardiac death compared to isolated volumetric parameters in patients with repaired Tetralogy of Fallot?
In patients with repaired Tetralogy of Fallot, integrating myocardial deformation (strain) and fibrosis markers (T1 mapping, ECV, LGE) with volumetric and electrophysiological data provides incremental prognostic value over isolated RV volumes for predicting ventricular arrhythmias and guiding pulmonary valve replacement timing.
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, and surgical advances now allow most patients to reach adulthood. However, anatomical repair does not eliminate long-term cardiovascular risk: chronic pulmonary regurgitation, frequently related to transannular patch repair, imposes volume overload on the right ventricle (RV) and triggers a dynamic remodeling process characterized by dilatation, altered geometry, progressive systolic and diastolic dysfunction, abnormal ventricular interaction and arrhythmogenic substrate formation. This article aims to narratively review the pathophysiological mechanisms of RV remodeling in repaired TOF, the main imaging assessment methods — echocardiography with strain analysis and cardiovascular magnetic resonance (CMR) with T1 mapping and late gadolinium enhancement — and their prognostic implications regarding ventricular arrhythmias and sudden cardiac death risk. The review indicates that isolated volumetric parameters, such as RV end-diastolic volume, have limited predictive value when used alone, whereas myocardial deformation measures (RV global longitudinal and free-wall strain) and fibrosis markers (extracellular volume, native T1 and late gadolinium enhancement) add incremental prognostic value. Contemporary risk stratification should integrate volumetric, functional, strain and fibrosis data with electrophysiological parameters to better guide the optimal timing of pulmonary valve replacement and reduce the risk of arrhythmic events and sudden death in this growing adult congenital heart disease population.
No takes yet. Share an insight, caveat, or question.
Boiko et al. (2026) conducted a review in Repaired Tetralogy of Fallot. Multimodal cardiac imaging (echocardiography with strain analysis and cardiovascular magnetic resonance with tissue characterization) vs. Isolated right ventricular volumetric parameters (such as RV end-diastolic volume) was evaluated on Ventricular arrhythmias and sudden cardiac death risk stratification. Myocardial strain and CMR fibrosis markers add incremental prognostic value over isolated RV volumes for predicting arrhythmias and sudden death in repaired Tetralogy of Fallot.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: