Magnetic resonance imaging in children: common problems and possible solutions for lung and airways imaging

Pediatric chest MRI is challenging. High-resolution scans of the lungs and airways are compromised by long imaging times, low lung proton density and motion. Low signal is a problem of normal lung. Lung abnormalities commonly cause increased signal intenstities. Among the most important factors for a successful MRI is patient cooperation, so the long acquisition times make patient preparation crucial. Children usually have problems with long breath-holds and with the concept of quiet breathing. Young children are even more challenging because of higher cardiac and respiratory rates giving motion blurring. For these reasons, CT has often been preferred over MRI for chest pediatric imaging. Despite its drawbacks, MRI also has advantages over CT, which justifies its further development and clinical use. The most important advantage is the absence of ionizing radiation, which allows frequent scanning for short- and long-term follow-up studies of chronic diseases. Moreover, MRI allows assessment of functional aspects of the chest, such as lung perfusion and ventilation, or airways and diaphragm mechanics. In this review, we describe the most common MRI acquisition techniques on the verge of clinical translation, their problems and the possible solutions to make chest MRI feasible in children

Lung MRI and impairment of diaphragmatic function in Pompe disease

Background: Pompe disease is a progressive metabolic myopathy. Involvement of respiratory muscles leads to progressive pulmonary dysfunction, particularly in supine position. Diaphragmatic weakness is considered to be the most important component. Standard spirometry is to some extent indicative but provides too little insight into diaphragmatic dynamics. We used lung MRI to study diaphragmatic and chest-wall movements in Pompe disease. Methods: In ten adult Pompe patients and six volunteers, we acquired two static spirometer-controlled MRI scans during maximum inspiration and expiration. Images were manually segmented. After normalization for lung size, changes in lung dimensions between inspiration and expiration were used for analysis; normalization was based on the cranial-caudal length ratio (representing vertical diaphragmatic displacement), and the anterior-posterior and left-right length ratios (representing chest-wall movements due to thoracic muscles). Results: We observed striking dysfunction of the diaphragm in Pompe patients; in some patients the diaphragm did not show any displacement. Patients had smaller cranial-caudal length ratios than volunteers (p < 0.001), indicating diaphragmatic weakness. This variable strongly correlated with forced vital capacity in supine position (r = 0.88) and postural drop (r = 0.89). While anterior-posterior length ratios also differed between patients and volunteers (p = 0.04), left-right length ratios did not (p = 0.1). Conclusions: MRI is an innovative tool to visualize diaphragmatic dynamics in Pompe patients and to study chest-walland diaphragmatic movements in more detail. Our data indicate that diaphragmatic displacement may be severely disturbed in patients with Pompe disease

Multiple common comorbidities produce left ventricular diastolic dysfunction associated with coronary microvascular dysfunction, oxidative stress, and myocardial stiffening

Aims More than 50% of patients with heart failure have preserved ejection fraction characterized by diastolic dysfunction. The prevalance of diastolic dysfunction is higher in females and associates with multiple comorbidities such as hypertension (HT), obesity, hypercholesterolemia (HC), and diabetes mellitus (DM). Although its pathophysiology remains incompletely understood, it has been proposed that these comorbidities induce systemic inflammation, coronary microvascular dysfunction, and oxidative stress, leading to myocardial fibrosis, myocyte stiffening and, ultimately, diastolic dysfunction. Here, we tested this hypothesis in a swine model chronically exposed to three common comorbidities.Methods and results DM (induced by streptozotocin), HC (produced by high fat diet), and HT (resulting from renal artery embolization), were produced in 10 female swine, which were followed for 6 months. Eight female healthy swine on normal pig-chow served as controls. The DM+HC+HT group showed hyperglycemia, HC, hypertriglyceridemia, renal dysfunction and HT, which were associated with systemic inflammation. Myocardial superoxide production was markedly increased, due to increased NOX activity and eNOS uncoupling, and associated with reduced NO production, and impaired coronary small artery endothelium-dependent vasodilation. These abnormalities were accompanied by increased myocardial collagen content, reduced capillary/fiber ratio, and elevated passive cardiomyocyte stiffness, resulting in an increased left ventricular end-diastolic stiffness (measured by pressure-volume catheter) and a trend towards a reduced E/A ratio (measured by cardiac MRI), while ejection fraction was maintained.Conclusions The combination of three common comorbidities leads to systemic inflammation, myocardial oxidative stress, and coronary microvascular dysfunction, which associate with myocardial stiffening and LV diastolic dysfunction with preserved ejection fraction

Lung morphology assessment using MRI: a robust ultra-short TR/TE 2D steady state free precession sequence used in cystic fibrosis patients

To evaluate feasibility and diagnostic quality of ultra-short TR/TE two-dimensional (2D) steady state free precession (SSFP) MRI for cystic fibrosis (CF) patients. We performed lung MRI at 1.5 Tesla in 20 CF-patients (6-17 years, 12 males). Axial, coronal, and sagittal sections were acquired in inspiration and expiration with maximum breath-hold time 10 s. MR and CT images were scored using a modified Brody scoring system to assess bronchiectasis, mucous plugging, atelectasis/consolidations, and air trapping. All images were scored by two experienced observers. A complete MR investigation took maximally 15 min. Maximal breath-holds were only 10 s and well tolerated. MRI identified major bronchiectasis, mucous plugging and atelectasis. End-expiratory scans showed patches of parenchyma with reduced signal intensity that may corresponded to areas of trapped air on expiratory CT scans. This MRI protocol based on ultra-short TR/TE 2D SSFP is quick and well tolerated and provides highly relevant imaging features as seen on CT in CF patients. Most importantly, the SNR of the expiratory scans enables to visualize air trapping. The preliminary results of this study suggest MRI as a noteworthy additional imaging tool for routine monitoring of CF patients

Imaging of respiratory muscles in neuromuscular disease: A review

Respiratory muscle weakness frequently occurs in patients with neuromuscular disease. Measuring respiratory function with standard pulmonary function tests provides information about the contribution of all respiratory muscles, the lungs and airways. Imaging potentially enables the study of different respiratory muscles, including the diaphragm, separately. In this review, we provide an overview of imaging techniques used to study respiratory muscles in neuromuscular disease. We identified 26 studies which included a total of 573 patients with neuromuscular disease. Imaging of respiratory muscles was divided into static and dynamic techniques. Static techniques comprise chest radiography, B-mode (brightness mode) ultrasound, CT and MRI, and are used to assess the position and thickness of the diaphragm and the other respiratory muscles. Dynamic techniques include fluoroscopy, M-mode (motion mode) ultrasound and MRI, used to assess diaphragm motion in one or more directions. We discuss how these imaging techniques relate with spirometric values and whether these can be used to study the contribution of the different respiratory muscles in patients with neuromuscular disease

Three-dimensional magnetic resonance coronary angiography using a new blood pool contrast agent:Initial experience

Objective: The aim of this feasibility study was to assess the effect of a new blood pool contrast agent on magnetic resonance coronary angiography (MRCA) in patients suspected of having coronary artery disease. Methods: Nine patients referred for diagnostic x-ray coronary, angiography in the evaluation of chest pain underwent MRCA using a thin-slab three-dimensional (3D) breath-hold segmented gradient echo technique at 1.5T before and after intravenous administration of feruglose, a new blood pool contrast agent. Results: A total of 36 coronary arteries, including 15 stenoses were evaluated. Overall agreement of visually assessed stenoses > 50% was 86%. The mean signal-to-noise ratio in the left anterior descending artery (LAD) did not change significantly after contrast administration, a relative improvement of 1.2 +/- 0.4 (p = 0.1), whereas the mean contrast-to-noise ratio of blood to myocardium improved by 3 +/- 2 (p = 0.01). Conclusion: A thin-slab 3D breath-hold scan combined with feruglose allows rapid localization and coverage of the proximal coronary arteries