Right ventricular dysfunction following tetralogy of Fallot correction: anatomical determinants and therapeutic strategies

Abstract

Right ventricular dysfunction following surgical correction of tetralogy of Fallot (TOF) remains a major determinant of long-term morbidity and mortality in survivors. Despite advancements in surgical techniques, residual anatomical abnormalities - including pulmonary regurgitation, right ventricular outflow tract obstruction, abnormal coronary artery anatomy, scar formation, and tricuspid regurgitation - synergistically drive ventricular remodeling and functional decline. This review synthesizes evidence on the pathophysiological interplay of these anatomical substrates. Key imaging modalities, such as 3D late gadolinium enhancement cardiac magnetic resonance, and artificial intelligence tools enhance risk stratification for ventricular arrhythmias. We propose a hierarchical management framework prioritizing hemodynamic stabilization, electrophysiological substrate modification and individualized strategies for concomitant lesions considerations. This work aims to bridge anatomical insights with therapeutic innovations, offering a roadmap for improving longevity and quality of life in repaired TOF patients.

Keywords: anatomical determinants; integrated management; right ventricular dysfunction; tetralogy of Fallot.

Figure 1.

An illustration showing the anatomical-pathophysiological interactions leading to RVD in rTOF patients.

Figure 2.

Multidisciplinary decision-making algorithm for multifactorial RVD in rTOF: integrating hemodynamic stratification, electrophysiological risk profiling, and precision therapeutics.