Related Experiment Video
Updated: May 11, 2026
Three-Dimensional Phase Resolved Functional Lung Magnetic Resonance Imaging
Published on: June 21, 2024
Aortopulmonary collateral flow in cystic fibrosis assessed with phase-contrast MRI
Robert Fleck1, Gary McPhail, Rhonda Szczesniak
1Department of Pediatric Radiology, Cincinnati Children's Hospital - MLC 5031, 3333 Burnett Avenue, Cincinnati, OH, 45229, USA, Robert.Fleck@cchmc.org.
Insights
Aortopulmonary collateral blood flow increases with cystic fibrosis (CF) severity, even in early stages. Phase-contrast MRI reliably detects this change, suggesting it as a sensitive biomarker for CF lung disease.
Area of Science:
- Cardiology
- Pulmonology
- Medical Imaging
Background:
- Cystic fibrosis (CF) is a common genetic disorder in Caucasians.
- Chronic pulmonary disease is a major CF morbidity.
- The link between CF clinical severity and aortopulmonary collateral blood flow is not well understood.
Purpose of the Study:
- To measure aortopulmonary collateral blood flow changes in children with CF.
- To assess these changes across the spectrum of CF disease severity.
- To utilize phase-contrast magnetic resonance imaging (MRI) for these measurements.
Main Methods:
- 16 CF patients were evaluated, categorized by FEV1% predicted (mild vs. moderate/severe).
- 17 healthy, age/gender-matched controls were included.
- Phase-contrast MRI quantified blood flow in the aorta and pulmonary arteries.
Main Results:
- Aortopulmonary collateral blood flow increased as FEV1% predicted decreased below 101.5%.
- Significantly higher collateral flow was observed in moderate/severe CF patients compared to controls (0.89 vs. 0.20 L/min).
- Collateral flow negatively correlated with FEV1% predicted (r=0.70, P=0.0050).
Conclusions:
- Phase-contrast MRI is a reliable tool for assessing aortopulmonary collateral blood flow in CF.
- Collateral flow begins to increase in mild CF, even with normal lung function.
- Elevated aortopulmonary collateral blood flow may be a sensitive biomarker for early pulmonary disease in CF.
Background:
Cystic fibrosis (CF) is a common genetic disease in Caucasians. Chronic pulmonary disease with progressive destruction of the pulmonary parenchyma is two of the major morbidities, but the relationship between clinical severity of CF and aortopulmonary collateral blood flow has not been assessed.
Objective:
The purpose of this study is to measure changes in aortopulmonary collateral blood flow by phase-contrast magnetic resonance imaging (MRI) in children with CF across the spectrum of disease severity as measured by the forced expiratory volume in one second as percent predicted value (FEV1% predicted).
Materials And Methods:
Sixteen patients with CF were prospectively evaluated. Eight were classified as having mild CF lung disease (FEV1 ≥80% predicted) and eight were classified as having moderate to severe CF lung disease (FEV1 <80% predicted). Seventeen age- and gender-matched non-CF subjects without cardiac or lung disease served as controls. Phase-contrast flow was measured at the ascending aorta, main pulmonary artery and both pulmonary arteries. Aortopulmonary collateral blood flow was calculated for each subject. The relationship between collateral flow and FEV1% predicted was modeled using nonparametric regression. Group differences were assessed by analysis of variance.
Results:
Aortopulmonary collateral blood flow began to increase as FEV1% predicted in subjects with CF fell below 101.5% with significant further increase in the aortopulmonary collateral blood flow in the subjects with CF with moderate to severe lung disease compared to controls (0.89 vs. 0.20 L/min, P < 0.0001). Aortopulmonary collateral blood flow correlated negatively with FEV1% predicted (r=0.70, P = 0.0050) confirming its relationship to this established marker of disease severity. There was no statistically significant difference in results obtained from two independent observers.
Conclusion:
These preliminary findings suggest that phase-contrast MRI can be performed reliably with consistent results and without interobserver variability. While the aortopulmonary collateral blood flow is within the normal range in subjects with mild CF disease, it begins to increase even when lung function is still in the normal range. A significant increase in the aortopulmonary collateral blood flow compared to controls is measured in patients with moderate to severe CF lung disease. The studies support the notion that aortopulmonary collateral blood flow may serve as a novel and sensitive biomarker of early pulmonary disease in cystic fibrosis.
