Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease with a growing population of adult survivors. Late pulmonary outflow tract and pulmonary valve postoperative complications are frequent, leading to long-term risks such as right heart failure and sudden death secondary to arrhythmias. Cardiac magnetic resonance imaging (CMR) is the gold standard for assessment of cardiac function in patients with repaired ToF. We aimed to determine the most useful CMR predictors of disease progression and the optimal frequency of CMR.

We systematically reviewed PubMed from inception until 29 April 2019 for longitudinal studies assessing the relationship between CMR features and disease progression in repaired ToF. Fourteen (14) studies were identified. Multiple studies showed that impaired right and left ventricular function predict subsequent disease progression. Right ventricular end diastolic volume, while being associated with disease progression when analysed alone, was generally not associated with disease progression on multivariate analysis. Severity of tricuspid regurgitation and pulmonary regurgitation likewise did not show a consistent association with subsequent events. A number of non-CMR factors were also identified as being associated with disease progression, in particular QRS duration and older age at repair. Restrictive right ventricular physiology was not consistently an independent predictor of events.

Impaired right and left ventricular function are the most consistent independent predictors of disease progression in repaired ToF. The optimal timing of repeat cardiac imaging remains controversial. Large scale prospective studies will provide important information to guide clinical decision making in this area.