Functional lung MRI for regional monitoring of patients with cystic fibrosis

Abstract

PurposeTo test quantitative functional lung MRI techniques in young adults with cystic fibrosis (CF) compared to healthy volunteers and to monitor immediate treatment effects of a single inhalation of hypertonic saline in comparison to clinical routine pulmonary function tests.Materials and methodsSixteen clinically stable CF patients and 12 healthy volunteers prospectively underwent two functional lung MRI scans and pulmonary function tests before and 2h after a single treatment of inhaled hypertonic saline or without any treatment. MRI-derived oxygen enhanced T1 relaxation measurements, fractional ventilation, first-pass perfusion parameters and a morpho-functional CF-MRI score were acquired.ResultsCompared to healthy controls functional lung MRI detected and quantified significantly increased ventilation heterogeneity in CF patients. Regional functional lung MRI measures of ventilation and perfusion as well as the CF-MRI score and pulmonary function tests could not detect a significant treatment effect two hours after a single treatment with hypertonic saline in young adults with CF (p>0.05).ConclusionThis study shows the feasibility of functional lung MRI as a non-invasive, radiation-free tool for monitoring patients with CF.

Assessment of the Reconstructed Pulmonary Circulation With Lung Perfusion Scintigraphy After Unifocalization and Repair of Tetralogy of Fallot With Major Aortopulmonary Collaterals

“…Although cardiac magnetic resonance imaging allows measurement of pulmonary blood flow with less radiation exposure than LPS, [26][27][28] and could be used instead of LPS, our practice over time has been to perform LPS. Ultimately, radiation exposure related to serial LPS in these patients should be considered in determining how often to image, in conjunction with ongoing assessment of the value of the perfusion data obtained.…”

Three-dimensional Isotropic Functional Imaging of Cystic Fibrosis Using Oxygen-enhanced MRI: Comparison with Hyperpolarized 3He MRI

“…Although these tools are important, they are problematic for longitudinal followup of pediatric patients because they lack sensitivity (1,2) and expose patients to radiation (3,4). Functional MRI techniques, such as hyperpolarized helium 3 ( 3 He) MRI (5)(6)(7)(8)(9) and, more recently, oxygen-enhanced (OE) MRI (10)(11)(12)(13), have been used to assess regional ventilation abnormalities of the lungs in patients with CF. Hyperpolarized 3 He MRI ventilation defect percentage (VDP) is an emerging biomarker in patients with obstructive lung disease (7,(14)(15)(16).…”

Cystic Fibrosis and Pseudomonas aeruginosa: the Host-Microbe Interface

“…upon radiological imaging than the lower lobes and left side of the lungs (197,198,(440)(441)(442)(443)(444)(445). Additionally, to the extent that techniques have measured regional lung function and ventilation/perfusion defects, the upper lobes are more compromised than the lower lobes (446,447). This interlobar heterogeneity has even prompted surgeons to perform lobectomies to selectively isolate and remove those regions of CF lungs that are most profoundly affected by the disease process (448,449).…”