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Lung findings of bronchopulmonary dysplasia on radiography and CT. a Anteroposterior (AP) chest radiograph of a premature infant girl born at 23 weeks of gestation, now post-menstrual age 38 weeks (15 weeks old). Portable chest radiograph shows diffuse increased density throughout the lungs but no evidence of air-trapping or cystic changes. b Coronal oblique minimum-intensity projection reconstructed CT image demonstrates hyperlucent lung in the left upper lobe and small cysts throughout the lungs. CT also suggested tracheomalacia was present (not shown)
Source publication
Bronchopulmonary dysplasia (BPD) is a common long-term complication of preterm birth. The chest radiograph appearance and survivability have evolved since the first description of BPD in 1967 because of improved ventilation and clinical strategies and the introduction of surfactant in the early 1990s. Contemporary imaging care is evolving with the...
Citations
... Magnetic resonance imaging (MRI) -method of choice to detect central nervous system pathology in the preterm population[23] -has gained increasing interest to qualitatively and quantitatively assess pulmonary disease [24,25] as well as to diagnose and monitor PH [26,27]. We successfully established an advanced MRI protocol that takes advantage of emerging techniques in phase-contrast (PC) MRI [28] in spontaneous sleep at near term age while avoiding the impact of sedation, oxygen (O2) supplementation or ventilatory support. We characterized MRI-based pulmonary artery (PA) flow and cardiac function in preterm infants with BPD-associated PVD and trained a PA flow model to synergistically explain variation in this cohort. ...
Rationale
Pulmonary vascular disease (PVD) affects the majority of preterm neonates with bronchopulmonary dysplasia (BPD) and significantly determines long-term mortality through undetected progression into pulmonary hypertension.
Objectives
To associate characteristics of pulmonary artery (PA) flow and cardiac function with BPD-associated PVD near term using advanced magnetic resonance imaging (MRI) for improved risk stratification.
Methods
Preterms <32 weeks postmenstrual age (PMA) with/without BPD were clinically monitored including standard echocardiography and prospectively enrolled for 3TMRI in spontaneous sleep near term (AIRR study). Semi-manual PA flow quantification (phase-contrast MRI, no BPD n=28, mild n=35, moderate/severe n=25) was complemented by cardiac function assessment (cine MRI).
Measurements and Main Results
We identified abnormalities in PA flow and cardiac function, i.e. increased net forward volume (ratio right-over-left), decreased mean relative area change and pathologic right end-diastolic volume to sensitively detect BPD-associated PVD while correcting for PMA (L1OAUC=0.88/sensitivity=0.80/specificity=0.81). We linked these changes to increased right ventricular (RV) afterload (RV-arterial coupling (p=0.02), PA midsystolic notching (p=0.015(t2)), cardiac index (p=1.67×10 ⁻⁸ )) and correlated echocardiographic findings. Identified in moderate/severe BPD, we successfully applied the PA flow model in heterogeneous mild BPD cases, demonstrating strong correlation of PVD probability with indicators of BPD severity, i.e., duration of mechanical ventilation (R=0.62, p=3.7×10 ⁻⁴ ) and oxygen supplementation (R=0.58, p=9.2×10 ⁻⁴ ).
Conclusions
Abnormalities in MRI PA flow and cardiac function exhibit significant, synergistic potential to detect BPD-associated PVD, advancing the possibilities of risk-adapted monitoring.
... The assessment of subglottic stenosis requires a flexible laryngoscopy or, more often, direct laryngoscopy with rigid bronchoscopy in order to accurately diagnose and grade the lesion [51]. Mild grades of subglottic stenosis are typically well tolerated; however, more severe grades may require tracheostomy placement and eventual airway reconstructive surgery when the infant is older and the airway is larger [52]. ...
Bronchopulmonary dysplasia (BPD), a disorder characterized by arrested lung development, is a frequent cause of morbidity and mortality in premature infants. Parenchymal lung changes in BPD are relatively well-characterized and highly studied; however, there has been less emphasis placed on the role that airways disease plays in the pathophysiology of BPD. In preterm infants born between 22 and 32 weeks gestation, the conducting airways are fully formed but still immature and therefore susceptible to injury and further disruption of development. The arrest of maturation results in more compliant airways that are more susceptible to deformation and damage. Consequently, neonates with BPD are prone to developing airway pathology, particularly for patients who require intubation and positive-pressure ventilation. Airway pathology, which can be divided into large and small airways disease, results in increased respiratory morbidity in neonates with chronic lung disease of prematurity.
... 24 Although plain chest radiography remains the mainstay of neonatal chest imaging and is always at the forefront of decision-making for infants with respiratory distress, it has a poor correlation with BPD severity. 25 The chest computed tomography (CT) scoring system may have higher objectivity and accuracy in the assessment of late respiratory outcomes 26,27 but is limited by the transportation of unstable infants to the CT scanner and concern about radiation. Chest magnetic resonance imaging (MRI) can evaluate lung tissue in infants with lung disease without radiation but is not always available in a NICU environment, and infants need to be sedated. ...
Objective:
Lung ultrasound (LUS) is a useful and radiation-free diagnostic tool for predicting bronchopulmonary dysplasia, which is a risk factor for late respiratory disease. However, data on the relationship of LUS with late respiratory disease was scarce. This study aims to determine whether LUS is associated with late respiratory disease during early childhood.
Methods:
This prospective cohort study enrolled preterm infants born before 32 weeks of gestation. LUS was performed at 36 weeks' postmenstrual age. The predictive values of a modified lung ultrasound (mLUS) score based on eight standard sections were assessed to predict late respiratory disease, defined as a physician diagnosis of bronchopulmonary dysplasia deterioration, asthma, reactive airway disease, bronchiolitis, pneumonia, or respiratory-related hospitalization during the first 2 years of life.
Results:
A total of 94 infants completed follow-up, of whom 74.5% met the late respiratory disease criteria. The mLUS scores were significantly associated with late respiratory disease (adjusted odds ratio: 1.23, CI: 1.10-1.38, p < 0.001). The mLUS scores also well predicted late respiratory disease (AUC = 0.820, 95% CI: 0.733-0.907). These scores were superior to the classic lung ultrasound score (p = 0.02) and as accurate as the modified NICHD-defined bronchopulmonary dysplasia classification (p = 0.91). A mLUS score ≥14 was the optimal cutoff point for predicting late respiratory disease.
Conclusion:
The modified lung ultrasound score correlates significantly with late respiratory disease and well predicts it in preterm infants during the first 2 years of life.
... BPD is characterised microscopically by simplified enlarged alveoli and, in severe cases, decreased pulmonary vascular development [5,7]. BPD is thought to be caused by a variety of antenatal and postnatal insults on the immature lung, clinically manifesting as a requirement for prolonged respiratory support and typical chest radiography findings of patchy hyperinflation with mixed areas of density and hyper-lucency [8]. ...
Pulmonary hypertension (PH) can develop in babies with bronchopulmonary dysplasia (BPD). PH is common in those with severe BPD and is associated with a high mortality rate. However, in babies surviving beyond 6 months, resolution of PH is likely. There is currently no standardised screening protocol for PH in BPD patients. Diagnosis in this group relies heavily on transthoracic echocardiography. Management of BPD-PH should be led by a multidisciplinary team and focus on optimal medical management of the BPD and associated conditions that may contribute to PH. PH-targeted pharmacotherapies have been used in BPD-PH. To date, these have not been investigated in clinical trials and evidence of their efficacy and safety is absent.
Educational aims
To identify those BPD patients most at risk of developing PH.
To be aware of detection, multidisciplinary management, pharmacological treatment and monitoring strategies for BPD-PH patients.
To understand the potential clinical course for patients with BPD-PH and that evidence on efficacy and safety of PH-targeted pharmacotherapy in BPD-PH is limited.
... Participants underwent a lung MRI to assess for average total proton density (at a lung volume of functional residual capacity plus 1 L), which is a marker of lung tissue inhomogeneity, and average proton density at full expiration, which is an exploratory surrogate measure of gas trapping. 19,24 Novel research has identified correlations between pulmonary function and proton density, [25][26][27] but MRI measures provide different information about lung architecture. 19 Complete methods for the MRI have been previously described. ...
... 35 Tests which evaluate the lung function or structure, such as pulmonary MRI, may provide us with new information for diagnosis and classification of prematurityassociated lung disease, which may correlate better with functional measures such as physical activity. 24,26,[36][37][38] Our study found that both lower daily step count and lower MVPA were correlated with diminished proton density at full expiration on MRI, a measure of gas trapping. In our previous PICTURE study, diminished MRI proton density at full expiration was correlated with PFT measures suggestive of gas trapping (higher RV/TLC and lower FEV1), 19 which have been shown to be associated with impaired exercise capacity in a previous study. ...
Introduction:
Children with a history of bronchopulmonary dysplasia (BPD) may have lower physical activity levels, but evidence to date is mixed. This study compared physical activity levels between children born extremely preterm with and without history of BPD, and examined their associations with pulmonary magnetic resonance imaging (MRI) and pulmonary function test (PFT) indices.
Methods:
This multi-centre cross-sectional study included children aged 7-9 years born extremely preterm, with and without BPD. Children wore a pedometer for one week, then completed the Physical Activity Questionnaire (PAQ), pulmonary MRI, and PFT. Spearman correlations and multivariable linear regression modelling were performed.
Results:
Of 45 children, 28 had a history of moderate-severe BPD. There were no differences in any physical activity outcomes by BPD status. Higher average daily step count and higher average daily moderate-to-vigorous physical activity (MVPA) were each correlated with greater forced vital capacity (r=0.41 and 0.58), greater MRI lung proton density at full expiration (r=0.42 and 0.49), and lower lung clearance index (r=-0.50 and -0.41). After adjusting for MRI total proton density and BPD status, a 5% increase in forced expiratory volume at one second was associated with 738 (95%CI: 208, 1268) more steps per day and 0.1 (0.0, 0.2) more hours of MVPA, respectively.
Conclusion:
School-aged children born extremely preterm have similar physical activity levels to their peers, regardless of history of BPD. MRI and PFT measures suggestive of gas trapping and/or airflow obstruction are associated with lower physical activity levels. This article is protected by copyright. All rights reserved.
... Our study is the first to assess functional neonatal lung ventilation defects by quantifying ventilation defect percentages in premature infants with BPD using free breathing phase-resolved functional lung MRI with a standard spoiled gradient echo sequence. Currently, neonatal lung imaging studies routinely focus only on structural abnormalities of lung parenchyma or tracheal airway using ultra-short echo time MRI or computed tomography (CT) [21][22][23]. However, quantitation of ventilation defects may provide additional quantitative biomarkers to those measured by structural sequences and to the respiratory support levels. ...
Background
The most common chronic complication of preterm birth is bronchopulmonary dysplasia (BPD), widely referred to as chronic lung disease of prematurity. All current definitions rely on characterizing the disease based on respiratory support level and do not provide full understanding of the underlying cardiopulmonary pathophysiology.Objective
To evaluate a rapid functional lung imaging technique in premature infants and to quantitate pulmonary ventilation using 1.5 Tesla magnetic resonance imaging (MRI).Materials and methodsWe conducted a prospective MRI study of 12 premature infants in the neonatal intensive care unit (NICU) using the phase resolved functional lung MRI technique to calculate pulmonary ventilation parameters in preterm infants with and without BPD grade 0/1 (n = 6) and grade 2/3 (n = 6).ResultsThe total ventilation defect percentage showed a significant difference between groups (16.0% IQR (11.0%,18%) BPD grade 2/3 vs. 8.0% IQR (4.5%,9.0%) BPD grade 0/1, p = 0.01).Conclusion
Phase-resolved functional lung MRI is feasible for assessment of ventilation defect percentages in preterm infants and shows regional variation in localized lung function in this population.
Respiratory disease is one of the most common complications of preterm birth. Survivors of prematurity have increased risks of morbidities and mortalities independent of prematurity, and frequently require multiple medications, home respiratory support, and subspecialty care to maintain health. Although advances in neonatal and pulmonary care have improved overall survival, earlier gestational age, lower birth weight, chorioamnionitis and late onset sepsis continue to be major factors in the development of bronchopulmonary dysplasia. These early life events associated with prematurity can have respiratory consequences that persist into adulthood. Furthermore, after initial hospital discharge, air pollution, respiratory tract infections and socioeconomic status may modify lung growth trajectories and influence respiratory outcomes in later life. Given that the incidence of respiratory disease associated with prematurity remains stable or increased, there is a need for pediatric and adult providers to be familiar with the natural history, manifestations, and common complications of disease.
The American Thoracic Society Core Curriculum updates clinicians annually in pediatric pulmonary disease. This is a summary of the Pediatric Pulmonary Medicine Core Curriculum presented at the 2023 American Thoracic Society International Conference. The respiratory disorders of infancy discussed in this year's review include: the care of the patient with bronchopulmonary dysplasia in the neonatal intensive care unit, clinical phenotypes and comorbidities; diffuse lung disease; pulmonary hypertension; central and obstructive sleep apnea. The care of infants with respiratory disorders often poses significant challenges to the general pediatric pulmonologist, sleep clinician, and neonatologist. This review aims to highlight the most clinically relevant aspects of the evaluation, management, and outcomes of infants with these key respiratory disorders, while emphasizing the importance of multidisciplinary care. Furthermore, this document summarizes essential aspects of genetic testing, novel imaging and treatment modalities, and includes multiple resources for clinical practice.
Introduction
It has recently been reported that it is possible to monitor lung oxygenation (rSO 2 L) by near‐infrared spectroscopy (NIRS) in preterm infants with respiratory distress syndrome (RDS). Thus, our aim was to assess the possibility of monitoring rSO 2 L in infants with evolving and established bronchopulmonary dysplasia (BPD) and to evaluate if rSO 2 L correlates with BPD severity and other oxygenation indices.
Methods
We studied 40 preterm infants with gestational age ≤30 weeks at risk for BPD. Patients were continuously studied for 2 h by NIRS at 28 ± 7 days of life and 36 weeks ± 7 days of postmenstrual age.
Results
rSO 2 L was similar at the first and second NIRS recordings (71.8 ± 7.2 vs. 71.4 ± 4.2%) in the overall population, but it was higher in infants with mild than in those with moderate‐to‐severe BPD at both the first (73.3 ± 3.1 vs. 71.2 ± 3.2%, p = .042) and second (72.3 ± 2.8 vs. 70.5 ± 2.8, p = .049) NIRS recording. A rSO 2 L cutoff value of 71.6% in the first recording was associated with a risk for moderate‐to‐severe BPD with a sensitivity of 66% and a specificity of 60%. Linear regression analysis demonstrated a significant positive relationship between rSO 2 L and SpO 2 /FiO 2 ratio ( p = .013) and a/APO 2 ( p = .004).
Conclusions
Monitoring of rSO 2 L by NIRS in preterm infants with evolving and established BPD is feasible and safe. rSO 2 L was found to be higher in infants with mild BPD, and predicts the risk for developing moderate‐to‐severe BPD and correlates with other indices of oxygenation.
Objective: To compare the accuracy of three newly proposed definitions of bronchopulmonary dysplasia (BPD) in predicting outcomes, and to assess the impact of BPD phenotypes (large airway vs. parenchymal vs. vascular disease) on BPD outcomes.
Study Design: Retrospective chart review of 100 infants with severe BPD discharged from a Children’s hospital between 2020-2021. Multivariable models evaluated the associations between BPD definitions and phenotypes with tracheostomy and death.
Result: Jensen’s and BPD collaborative criteria best predicted outcomes associated with tracheostomy and/or death (p < 0.001). Among the three BPD phenotypes, large airway disease independently predicted death or tracheostomy (OR 10.5, 95% CI 1.6, 68.1). The combination of all three phenotypes also predicted death or tracheostomy (OR 9.8, 95% CI 1.0, 93.5).
Conclusion: Newly proposed definitions of BPD better predict outcomes compared to the 2001 NIH definition with BPD phenotypes impacting mortality and short-term outcomes. These data may be useful for counseling families and developing phenotype-based individualized treatment plans.
