Non-contrast Lung Perfusion Mapping Applied for New Insights in Cystic Fibrosis

• Status: Recruiting
• Trial end date: 2023-08-01
• Target enrollment: 0
• Participant gender: All

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.

• Phase: Phase 4
• Accepts Healthy Volunteers?: Accepts Healthy Volunteers

Details

• Lead Sponsor: Children's Hospital Medical Center, Cincinnati
• Treatments: Xenon

Criteria

Inclusion Criteria:

Inclusion CF Cohort

• male or female between the ages of 6 through 21 years

• diagnosis of CF by positive sweat test and genetic test

• planning to start Trikafta based on clinical decision

• baseline pulmonary function test (PFT) defined as FEV1% that is no less than 5% of the best PFT in the previous 6 months

• Absence of exacerbation defined as

• No acute antibiotic usage for 14 days prior to MRI visit

• Able to perform an acceptable and reproducible spirometry

• O2 saturation level at 90% or greater when laying flat

Inclusion Healthy Control Cohort

• male or female between the ages of 6 through 21 years

• no known diagnoses that impact lung function in the opinion of the investigators

Exclusion Criteria:

Exclusion both cohorts

• standard MRI exclusions (metal implants, claustrophobia)

• pregnancy