Late gadolinium enhancement in patients with Tetralogy of Fallot: A systematic review

Purpose: The aim of this study is to review the literature concerning myocardial late gadolinium enhancement (LGE) with cardiac magnetic resonance in patients with Tetralogy of Fallot (ToF), with regards to its prevalence, characteristics and clinical relevance. Methods: We performed a systematic search, aiming to retrieve original articles that evaluated LGE in ToF, running a search string on MEDLINE and EMBASE in November 2019 and November 2020. Papers were then selected by two independent, blinded readers based on title and abstract, and then on full-text reading, and articles which did not include LGE evaluation were excluded. From each included paper two readers extracted descriptive data concerning technical parameters of LGE acquisition, LGE description and clinical significance. Results: 18 articles were eventually included in our review. The included studies observed that a higher amount of right ventricular LGE relates with higher right ventricular volumes, lower ejection fraction and a higher pulmonary regurgitant fraction, thus acting as a marker of progressive impairment of myocardial function. Moreover, LGE in ToF patients correlated with the onset of arrhythmias, and with serum biomarkers indicative of myocardial stress and fibrosis. Conclusions: LGE could be used in the follow-up repaired ToF patients as its appraisal can provide information concerning cardiac dysfunction. Moreover, it may be ideal to aim towards a common framework for standardizing assessment and quantification of LGE in ToF patients.

Automated summary

This study reviewed literature on myocardial late gadolinium enhancement in Tetralogy of Fallot patients, finding that higher amounts of right ventricular LGE are associated with worsening cardiac function, arrhythmias, and myocardial stress and fibrosis biomarkers.