Assessment of Disease Progression in Patients With Repaired Tetralogy of Fallot Using Cardiac Magnetic Resonance Imaging: A Systematic Review

Heart Lung Circ. 2020 Nov;29(11):1613-1620. doi: 10.1016/j.hlc.2020.04.017. Epub 2020 Jul 3.

Abstract

Aims: 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.

Methods and results: 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.

Conclusion: 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.

Keywords: Cardiac magnetic resonance imaging; Pulmonary valve replacement; Tetralogy of Fallot.

Publication types

  • Systematic Review

MeSH terms

  • Disease Progression
  • Electrocardiography
  • Humans
  • Magnetic Resonance Imaging, Cine / methods*
  • Tetralogy of Fallot / diagnosis*
  • Tetralogy of Fallot / physiopathology
  • Ventricular Function, Right / physiology*