Non-contrast Lung Perfusion Mapping Applied for New Insights in Cystic Fibrosis
Status:
Recruiting
Trial end date:
2023-08-01
Target enrollment:
Participant gender:
Summary
Cystic fibrosis (CF) results in the thickening of mucus in the lungs and other organs due to
dysfunction of a transmembrane conductance protein. This allows buildup of bacteria that
results in inflammation, leading to tissue breakdown and loss of function. In the lungs, this
process causes loss of air exchange structures progressing to diminished lung function. The
exchange of oxygen in the lungs depends on both the integrity of air conduits and
vasculature. Most clinical assessments, however, focus on ventilatory function, with the
assumption that any vascular compromise is secondary. Nevertheless, there is evidence, some
from the investigator's lab, to suggest that perfusion anomalies in the lung occur before
signs of ventilatory dysfunction. Thus, the inflammatory processes of CF may impact pulmonary
microvasculature specifically and concurrently or prior to damage to ventilatory structures.
This study aims to apply a new MRI method to serially measure regional lung perfusion,
without the use of contrast agent, in children with CF and to associate it with regional
assessments of ventilation and to serum cytokines or proteomic markers of angiogenesis and
inflammatory processes.
The investigator's lab has recently developed a noninvasive, non-contrast, method of labeling
blood flowing into the lungs and generating a map of perfusion. The investigator aims to
couple this technique to existing methods using hyperpolarized Xenon to map ventilation. The
investigator will apply these methods over time in CF patients, monitoring the relationship
between regional perfusion and ventilation defects.
This pilot work will provide the foundation for larger studies to establish the essential
etiological role of perfusion deficits in CF.