Pediatric Radiology

, Volume 43, Issue 10, pp 1279–1286

Aortopulmonary collateral flow in cystic fibrosis assessed with phase-contrast MRI

  • Robert Fleck
  • Gary McPhail
  • Rhonda Szczesniak
  • Joshua Knowlton
  • Rupa Radhakrishnan
  • John Clancy
  • Raouf Amin
Original Article

DOI: 10.1007/s00247-013-2708-z

Cite this article as:
Fleck, R., McPhail, G., Szczesniak, R. et al. Pediatr Radiol (2013) 43: 1279. doi:10.1007/s00247-013-2708-z

Abstract

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.

Keywords

Phase-contrast MRI Cystic fibrosis Aortopulmonary collateral blood flow FEV1 Cardiac Pulmonary hypertension Bronchosystemic shunt Children 

Abbreviations

APCBF

Aortopulmonary collateral blood flow

ANOVA

Analysis of variance

CF

Cystic fibrosis

CI

95% confidence interval

FEV1%

Forced expiratory volume in one second (percent predicted)

FEF25–75%

Forced expiratory flow25–75% (percent predicted)

FVC

Forced vital capacity

ICC

Intraclass correlation coefficient

Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • Robert Fleck
    • 1
  • Gary McPhail
    • 2
  • Rhonda Szczesniak
    • 3
  • Joshua Knowlton
    • 4
  • Rupa Radhakrishnan
    • 5
  • John Clancy
    • 2
  • Raouf Amin
    • 2
  1. 1.Department of Pediatric RadiologyCincinnati Children’s Hospital – MLC 5031CincinnatiUSA
  2. 2.Department of Pulmonary MedicineCincinnati Children’s HospitalCincinnatiUSA
  3. 3.Division of Biostatistics and EpidemiologyCincinnati Children’s HospitalCincinnatiUSA
  4. 4.School of Medicine, Children’s Mercy Hospital and ClinicsUniversity of Missouri at Kansas CityKansas CityUSA
  5. 5.Department of RadiologyUniversity of Cincinnati College of MedicineCincinnatiUSA

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