Neonatal Outcomes of Multiple Congenital Thoracic Lesions.

INTRODUCTION: Congenital pulmonary airway malformations (CPAM), intra- and extralobar bronchopulmonary sequestrations (iBPS/eBPS), CPAM-BPS hybrid lesions (HL), congenital lobar emphysema (CLE), bronchial atresia (BA), and foregut duplication cysts (FDC), collectively referred to as congenital thoracic lesions (CTL), are mostly solitary. Patients with multiple CTL are rare, and reports on such cases are scarce. To address this dearth, we analyzed a large multifocal CTL patient cohort. METHODS: Retrospective chart review of patients born between 1/1/2013 and 3/31/2023 who underwent surgery for a CTL at our tertiary center. Patients with radiological and surgical diagnosis of multifocal CTL, defined as ≥2 CTL present in more than one lobe were included to record pre-, peri-, and postnatal patient characteristics. RESULTS: Among 701 CTL patients, 74(10.5%) had multiple CTL. CTL multifocality was prenatally recognized correctly in 8(12.9%) patients. Most multiple CTL were right-sided, unilateral multilobar lesions (n=33, 44%). Bilateral CTL were found in 9(12.1%) patients. CPAM-CPAM lesions were the most prevalent CTL types (n=36, 49%). Genetic syndromes were confirmed in 3(4%) and additional congenital anomalies in 9(12.9%) patients, 5 of those had multiple congenital anomalies. Of 49(65%) patients with multilobar CTL, 25(51%) underwent bilobectomy and 24(49%) lung-sparing surgery. Length of stay was similar. Mortality was 5.4%. CONCLUSION: We report on the largest patient cohort with multiple CTL to date. Multiple CTL occurred in 1/10 patients with CTL, and only 12.9% were recognized prenatally. Lung-sparing surgery can be considered. Multiple additional congenital anomalies and genetic syndromes may be more common and genetic testing should be considered. Overall, outcomes in this patient population are favorable.

Surfactant treatment at birth in a contemporary cohort of preterm infants with bronchopulmonary dysplasia.

OBJECTIVE: Initial surfactant studies demonstrated improvements in survival and need for respiratory support. However, as the use of non-invasive respiratory support has increased the use of surfactant has decreased. We examined in a contemporary cohort of BPD patients if surfactant use was associated with BPD severity. STUDY DESIGN: An observational study using data from the BPD Collaborative Registry. RESULTS: 971 infants with BPD met entry criteria, 864 (89%) had received surfactant in the first 72 h of life (SURF) and the remainder had not (no surfactant). There was an association between SURF and BPD grade, with a greater likelihood of grade 3 BPD in infants who received surfactant in the DR or who had 2 or more doses. CONCLUSIONS: We speculate that the use of surfactant in the DR and use of multiple doses reflect the impact of perinatal factors beyond immaturity alone that increase the risk for grade 3 BPD.

Lived experiences of caregivers with infants treated at home with continuous invasive or noninvasive ventilation.

RATIONALE: Caring for children dependent upon continuous invasive ventilation in the home setting requires extensive expertise, coordination, and can result in impaired caregiver quality of life. Less is known regarding the experiences of caregivers with children requiring continuous noninvasive ventilation. OBJECTIVES: To evaluate caregiver experiences with invasive and noninvasive home mechanical ventilation, and to compare parental quality of life based on the child's mode of ventilation. METHODS: Caregivers of infants who were discharged home with continuous ventilatory support were recruited to complete semi-structured qualitative interviews. Interviews explored their decision-making process, the transition to home, and health related quality of life. RESULTS: Caregivers of 16 children were interviewed, of whom eight were treated with continuous invasive ventilation, and eight with continuous noninvasive ventilation. The decision to pursue home ventilation in both groups was greatly influenced by the desire to be discharged and reunite the family at home. Following the transition to home, caregivers from both groups described high rates of insomnia, emotional distress, work disruption and familial hardship. Despite this, parents were overwhelmingly pleased with the decision to proceed with home ventilation and perceived their children to be living enriched lives outside of the hospital. Factors associated with easing the transition to home were in-hospital training and the presence of a robust support system. CONCLUSIONS: Lives of families with children dependent on continuous mechanical ventilation are characterized by isolation, lifestyle disruption, adverse mental and physical health consequences, and impaired interpersonal relationships, irrespective of the mode of ventilation.

Update on Management and Outcomes of Congenital Diaphragmatic Hernia.

Infants with congenital diaphragmatic hernia (CDH) benefit from comprehensive multidisciplinary teams that have experience in caring for the unique and complex issues associated with CDH. Despite prenatal referral to specialized high-volume centers, advanced ventilation strategies and pulmonary hypertension management, and extracorporeal membrane oxygenation, mortality and morbidity remain high. These infants have unique and complex issues that begin in fetal and infant life, but persist through adulthood. Here we will review the literature and share our clinical care pathway for neonatal care and follow up. While many advances have occurred in the past few decades, our work is just beginning to continue to improve the mortality, but also importantly the morbidity of CDH.

Respiratory function after birth in infants with congenital diaphragmatic hernia.

OBJECTIVE: To characterise the transitional pulmonary physiology of infants with congenital diaphragmatic hernia (CDH) using measures of expiratory tidal volume (TV) and end-tidal carbon dioxide (ETCO2). DESIGN: Prospective single-centre observational study. SETTING: Quaternary neonatal intensive care unit. PATIENTS: Infants with an antenatal diagnosis of CDH born at the Children's Hospital of Philadelphia. INTERVENTIONS: TV and ETCO2 were simultaneously recorded using a respiratory function monitor (RFM) during invasive positive pressure ventilation immediately after birth. MAIN OUTCOME MEASURES: TV per birth weight and ETCO2 values were summarised for each minute after birth. Subgroups of interest were defined by liver position (thoracic vs abdominal) and extracorporeal membrane oxygenation (ECMO) treatment. RESULTS: RFM data were available for 50 infants from intubation until a median (IQR) of 9 (7-14) min after birth. TV and ETCO2 values increased for the first 10 min after birth, but intersubject values were heterogeneous. TVs were overall lower and ETCO2 values higher in infants with an intrathoracic liver and infants who were ultimately treated with ECMO. On hospital discharge, survival was 88% (n=43) and 34% (n=17) of infants were treated with ECMO. CONCLUSION: Respiratory function immediately after birth is heterogeneous for infants with CDH. Lung aeration, as evidenced by expired TV and ETCO2, appears to be ongoing throughout the first 10 min after birth during invasive positive pressure ventilation. Close attention to expired TV and ETCO2 levels by 10 min after birth may provide an opportunity to optimise and individualise ventilatory support for this high-risk population.

Use of ventilation/perfusion mismatch to guide individualised CPAP level selection in preterm infants: a feasibility trial.

OBJECTIVE: To measure within-subject changes in ventilation/perfusion (V'/Q') mismatch in response to a protocol of individualised nasal continuous positive airway pressure (CPAP) level selection. DESIGN: Single-arm, non-randomised, feasibility trial. SETTING: Three centres in the Children's Hospital of Philadelphia neonatal care network. PATIENTS: Twelve preterm infants of postmenstrual age 27-35 weeks, postnatal age >24 hours, and receiving a fraction of inspired oxygen (FiO2) >0.25 on CPAP of 4-7 cm H2O. INTERVENTIONS: We applied a protocol of stepwise CPAP level changes, with the overall direction and magnitude guided by individual responses in V'/Q' mismatch, as determined by the degree of right shift (kilopascals, kPa) in a non-invasive gas exchange model. Best CPAP level was defined as the final pressure level at which V'/Q' improved by more than 5%. MAIN OUTCOME MEASURES: Within-subject change in V'/Q' mismatch between baseline and best CPAP levels. RESULTS: There was a median (IQR) within-subject reduction in V'/Q' mismatch of 1.2 (0-3.2) kPa between baseline and best CPAP levels, p=0.02. Best CPAP was observed at a median (range) absolute level of 7 (5-8) cm H2O. CONCLUSIONS: Non-invasive measures of V'/Q' mismatch may be a useful approach for identifying individualised CPAP levels in preterm infants. The results of our feasibility study should be interpreted cautiously and replication in larger studies evaluating the impact of this approach on clinical outcomes is needed. TRIAL REGISTRATION NUMBER: NCT02983825.

Pediatric Simulation of Intrinsic PEEP and Patient-Ventilator Trigger Asynchrony During Mechanical Ventilation.

BACKGROUND: Intrinsic PEEP during mechanical ventilation occurs when there is insufficient time for expiration to functional residual capacity before the next inspiration, resulting in air trapping. Increased expiratory resistance (RE), too rapid of a patient or ventilator breathing rate, or a longer inspiratory to expiratory time ratio (TI/TE) can all be causes of intrinsic PEEP. Intrinsic PEEP can result in increased work of breathing and patient-ventilator asynchrony (PVA) during patient-triggered breaths. We hypothesized that the difference between intrinsic PEEP and ventilator PEEP acts as an inspiratory load resulting in trigger asynchrony that needs to be overcome by increased respiratory muscle pressure (Pmus). METHODS: Using a Servo lung model (ASL 5000) and LTV 1200 ventilator in pressure control mode, we developed a passive model demonstrating how elevated RE increases intrinsic PEEP above ventilator PEEP. We also developed an active model investigating the effects of RE and intrinsic PEEP on trigger asynchrony (expressed as percentage of patient-initiated breaths that failed to trigger). We then studied if trigger asynchrony could be reduced by increased Pmus. RESULTS: Intrinsic PEEP increased significantly with increasing RE (r = 0.97, P = .006). Multivariate logistic regression analysis showed that both RE and negative Pmus levels affect trigger asynchrony (P < .001). CONCLUSIONS: A passive lung model describes the development of increasing intrinsic PEEP with increasing RE at a given ventilator breathing rate. An active lung model shows how this can lead to trigger asynchrony since the Pmus needed to trigger a breath is greater with increased RE, as the inspiratory muscles must overcome intrinsic PEEP. This model will lend itself to the study of intrinsic PEEP engendered by a higher ventilator breathing rate, as well as higher TI/TE, and will be useful in ventilator simulation scenarios of PVA. The model also suggests that increasing ventilator PEEP to match intrinsic PEEP can improve trigger asynchrony through a reduction in RE.

Association of Racial Disparities With In-Hospital Outcomes in Severe Bronchopulmonary Dysplasia.

Importance: Bronchopulmonary dysplasia (BPD) is the most common serious morbidity of preterm birth. Short-term respiratory outcomes for infants with the most severe forms of BPD are highly variable. The mechanisms that explain this variability remain unknown and may be mediated by racial disparities. Objective: To determine the association of maternal race with death and length of hospital stay in a multicenter cohort of infants with severe BPD. Design, Setting, and Participants: This multicenter cohort study included preterm infants enrolled in the BPD Collaborative registry from January 1, 2015, to July 19, 2021, involving 8 BPD Collaborative centers located in the US. Included patients were born at less than 32 weeks' gestation, had a diagnosis of severe BPD as defined by the 2001 National Institutes of Health Consensus Criteria, and were born to Black or White mothers. Exposures: Maternal race: Black vs White. Main Outcomes and Measures: Death and length of hospital stay. Results: Among 834 registry infants (median [IQR] gestational age, 25 [24-27] weeks; 492 male infants [59%]) meeting inclusion criteria, the majority were born to White mothers (558 [67%]). Death was observed infrequently in the study cohort (32 [4%]), but Black maternal race was associated with an increased odds of death (adjusted odds ratio, 2.1; 95% CI, 1.2-3.5) after adjusting for center. Black maternal race was also significantly associated with length of hospital stay (adjusted between-group difference, 10 days; 95% CI, 3-17 days). Conclusions and Relevance: In a multicenter severe BPD cohort, study results suggest that infants born to Black mothers had increased likelihood of death and increased length of hospital stay compared with infants born to White mothers. Prospective studies are needed to define the sociodemographic mechanisms underlying disparate health outcomes for Black infants with severe BPD.

Pulmonary function tests in extremely low gestational age infants at one year of age.

RATIONALE: Identifying neonatal and post-discharge exposures among extremely low gestational age newborns (ELGANs) that drive increased pulmonary morbidity and abnormal lung function at 1 year of age proves challenging. OBJECTIVE: The NIH-sponsored Prematurity and Respiratory Outcomes Program (PROP), evaluated infant pulmonary function tests (iPFTs) at 1 year corrected age to determine which demographic and clinical factors are associated with abnormal lung function. METHODS: iPFTs were performed on a PROP subcohort of 135 participants following Institutional Review Board (IRB)-approved written consent. Demographic data, Neonatal Intensive Care Unit (NICU) clinical care, and post-NICU exposures were analyzed for association with iPFTs. MAIN RESULTS: A significant decrease in forced expiratory volume at 0.5 s (FEV0.5 ) and/or forced expiratory flows at 75% of forced vital capacity (FEF75 ), were associated with male sex and African American race. Clinical factors including longer duration of ventilatory support, exposure to systemic steroids, and weight less than the 10th percentile at 36 weeks postmenstrual age were also associated with airflow obstruction, whereas supplemental oxygen requirement and bronchopulmonary dysplasia were not. Additionally, the need for respiratory medications, technology, or hospitalizations during the first year, ascertained by a quarterly survey, were the only post-NICU factors associated with decreased FEV0.5 and FEF75 . Only 7% of infants had reversible airflow obstruction. CONCLUSIONS: Neonatal demographic factors, respiratory support in the NICU, and a history of greater post-NICU medical utilization for respiratory disease had the strongest association with lower lung function at 1 year in ELGANs.

Outpatient Respiratory Management of Infants, Children, and Adolescents with Post-Prematurity Respiratory Disease: An Official American Thoracic Society Clinical Practice Guideline.

Background: Premature birth affects millions of neonates each year, placing them at risk for respiratory disease due to prematurity. Bronchopulmonary dysplasia is the most common chronic lung disease of infancy, but recent data suggest that even premature infants who do not meet the strict definition of bronchopulmonary dysplasia can develop adverse pulmonary outcomes later in life. This post-prematurity respiratory disease (PPRD) manifests as chronic respiratory symptoms, including cough, recurrent wheezing, exercise limitation, and reduced pulmonary function. This document provides an evidence-based clinical practice guideline on the outpatient management of infants, children, and adolescents with PPRD. Methods: A multidisciplinary panel of experts posed questions regarding the outpatient management of PPRD. We conducted a systematic review of the relevant literature. The Grading of Recommendations, Assessment, Development, and Evaluation approach was used to rate the quality of evidence and the strength of the clinical recommendations. Results: The panel members considered the strength of each recommendation and evaluated the benefits and risks of applying the intervention. In formulating the recommendations, the panel considered patient and caregiver values, the cost of care, and feasibility. Recommendations were developed for or against three common medical therapies and four diagnostic evaluations in the context of the outpatient management of PPRD. Conclusions: The panel developed recommendations for the outpatient management of patients with PPRD on the basis of limited evidence and expert opinion. Important areas for future research were identified.

Predicting neonatal outcomes in infants with giant omphalocele using prenatal magnetic resonance imaging calculated observed-to-expected fetal lung volumes.

OBJECTIVE: To examine the association between prenatal magnetic resonance imaging (MRI) based observed/expected total lung volume (O/E TLV) and outcome in neonates with giant omphalocele (GO). METHODS: Between 06/2004 and 12/2019, 67 cases with isolated GO underwent prenatal and postnatal care at our institution. MRI-based O/E TLVs were calculated based on normative data from Meyers and from Rypens and correlated with postnatal survival and morbidities. O/E TLV scores were grouped based on severity into <25% (severe), between 25% and 50% (moderate), and >50% (mild) for risk stratification. RESULTS: O/E TLV was calculated for all patients according to Meyers nomograms and for 49 patients according to Rypens nomograms. Survival for GO neonates with severe, moderate, and mild pulmonary hypoplasia based on Meyers O/E TLV categories was 60%, 92%, and 96%, respectively (p = 0.04). There was a significant inverse association between Meyers O/E TLV and risk of neonatal morbidities (p < 0.05). A similar trend was observed with Rypens O/E TLV, but associations were less often significant likely related to the smaller sample size. CONCLUSION: Neonatal outcomes are related to fetal lung size in isolated GO. Assessment of Meyers O/E TLV allows identification of GO fetuses at greatest risk for complications secondary to pulmonary hypoplasia.

Invasive mechanical ventilation at 36 weeks post-menstrual age, adverse outcomes with a comparison of recent definitions of bronchopulmonary dysplasia.

OBJECTIVES: To determine whether the need for invasive mechanical ventilation (iMV) at 36 weeks PMA in patients with severe bronchopulmonary dysplasia (sBPD) identifies those patients at highest risk for tracheostomy or gastrostomy, and to compare sBPD with recent definitions of BPD. STUDY DESIGN: Observational study from Jan 2015 to Sept 2019 using data from the BPD Collaborative Registry. RESULTS: Five hundred and sixty-four patients with sBPD of whom 24% were on iMV at 36 weeks PMA. Those on iMV had significantly (p < 0.0001) increased risk for tracheostomy or gastrostomy. The overall mortality rate was 3% and the risk for mortality was substantially greater in those on iMV than in those on noninvasive support at 36 weeks PMA (RR 13.8, 95% CI 4.3-44.5, p < 0.0001). When applying the NICHD definition (2016) 44% had Grade III BPD. When applying the NRN definition, 6% had Grade 1 BPD, 70% had Grade 2 BPD, and 24% had Grade 3 BPD. CONCLUSIONS: Patients with sBPD who were on iMV at 36 weeks had a significantly greater risk of inhospital mortality and survivors had a significantly greater risk of undergoing tracheostomy and/or gastrostomy. The use of type 2 sBPD or Grade 3 BPD would enhance the ability to target future studies to those infants with sBPD at the highest risk of adverse long-term outcomes.

Established severe BPD: is there a way out? Change of ventilatory paradigms.

Improved survival of extremely preterm newborn infants has increased the number of infants at risk for developing bronchopulmonary dysplasia (BPD). Despite efforts to prevent BPD, many of these infants still develop severe BPD (sBPD) and require long-term invasive mechanical ventilation. The focus of research and clinical management has been on the prevention of BPD, which has had only modest success. On the other hand, research on the management of the established sBPD patient has received minimal attention even though this condition poses large economic and health problems with extensive morbidities and late mortality. Patients with sBPD, however, have been shown to respond to treatments focused not only on ventilatory strategies but also on multidisciplinary approaches where neurodevelopmental support, growth promoting strategies, and aggressive treatment of pulmonary hypertension improve their long-term outcomes. In this review we will try to present a physiology-based ventilatory strategy for established sBPD, emphasizing a possible paradigm shift from acute efforts to wean infants at all costs to a more chronic approach of stabilizing the infant. This chronic approach, herein referred to as chronic phase ventilation, aims at allowing active patient engagement, reducing air trapping, and improving ventilation-perfusion matching, while providing sufficient support to optimize late outcomes. IMPACT: Based on pathophysiological aspects of evolving and established severe BPD in premature infants, this review presents some lung mechanical properties of the most severe phenotype and proposes a chronic phase ventilatory strategy that aims at reducing air trapping, improving ventilation-perfusion matching and optimizing late outcomes.

Bronchopulmonary dysplasia-A historical perspective.

Bronchopulmonary dysplasia (BPD) was first described by Northway et al in 1967. This article describes the evolution of our understanding of the pathophysiology of BPD and the approaches to treatments of this illness developed over the past fifty years. These interventions had their roots in the understanding of the principles of the surface tension present at air-liquid interfaces, which were developed over 150 years before BPD's initial description. Improving outcomes in neonatal care have led to greater survival of preterm and very preterm infants, and to an evolution of the pathogenesis and pathology of BPD, from an illness caused primarily by barotrauma and oxygen toxicity to one of interruption of lung development. While the incidence of BPD has remained about the same in recent decades, this is because survival of infants born at lower gestational ages is increasing. Understanding of molecular, genetic and physiologic mechanisms has led to newer treatments that have mitigated some of the harmful effects of prolonged mechanical ventilation. Recognition of BPD as a chronic multi-system disease has resulted in further improvements in care after discharge from neonatal intensive care. Since many of the origins of chronic obstructive lung disease in adults are based in childhood respiratory illnesses, improving outcomes of BPD in infancy and childhood will undoubtedly lead to improved respiratory outcomes in the adults that these children will become.

Tolerability and efficacy of two doses of aerosolized albuterol in ventilated infants with BPD: A randomized controlled crossover trial.

RATIONALE: Aerosolized albuterol is widely used, but its tolerability and efficacy in infants with severe bronchopulmonary dysplasia (sBPD) is not well established. OBJECTIVES: To compare the tolerability and efficacy of two dose levels of aerosolized albuterol to saline placebo in infants with sBPD. METHODS: Single-center, multiple-crossover trial in 24 ventilated very preterm infants with sBPD. Albuterol (1.25 mg, 2.5 mg) and 3 ml of normal saline were administered every 4 h during separate 24-h treatment periods assigned in random order with a 6-h washout phase between periods. The primary outcome was the absolute change (post and pretherapy) in expiratory flow at 75% of exhalation (EF75). Secondary endpoints were changes in ventilator parameters, vital signs, and heart arrhythmia. RESULTS: Average within subject EF75 values improved with each therapy: saline placebo ( + 0.45 L/min ± 2.5, p = .04), 1.25 mg of albuterol ( + 0.70 L/min ± 2.4, p < .001), and 2.5 mg of albuterol ( + 0.38 L/min ± 2.4, p = .06). However, 1.25 mg of albuterol (0.26 L/min; 95% CI -0.19, 0.72) and 2.5 mg (-0.10 L/min; 95% CI -0.77, 0.57) produced similar changes in EF75 when compared to saline. All secondary outcomes were similar between saline and 1.25 mg of albuterol. Peak inspiratory pressure needed to deliver goal tidal volumes (7.5% relative decrease, 95% CI 2.6, 12.3) and heart rate (6.5% increase, 95% CI 2.2, 10.8) differed significantly between albuterol 2.5 mg and saline. CONCLUSION: Albuterol at 1.25 mg and 2.5 mg, compared to aerosolized saline, did not affect EF75 in infants with sBPD receiving invasive ventilation. Greater improvement in peak inspiratory pressures with albuterol 2.5 mg suggests benefit, but close heart monitoring may be indicated.

Pulmonary Manifestations of Congenital Heart Disease in Children.

This review addresses how anomalous cardiovascular anatomy imparts consequences to the airway, respiratory system mechanics, pulmonary vascular system, and lymphatic system. Abnormal formation or enlargement of great vessels can compress airways and cause large and small airway obstructions. Alterations in pulmonary blood flow associated with congenital heart disease (CHD) can cause abnormalities in pulmonary mechanics and limitation of exercise. CHD can lead to pulmonary arterial hypertension. Lymphatic abnormalities associated with CHD can cause pulmonary edema, chylothorax, or plastic bronchitis. Understanding how the cardiovascular system has an impact on pulmonary growth and function can help determine options and timing of intervention.

Development and validation of a novel informational booklet for pediatric long-term ventilation decision support.

OBJECTIVES: To provide accessible, uniform, comprehensive, and balanced information to families deciding whether to initiate long-term ventilation (LTV) for their child, we sought to develop and validate a novel informational resource. METHODS: The Ottawa Decision Support Framework was followed. Previous interviews with 44 lay and 15 professional stakeholders and published literature provided content for a booklet. Iterative versions were cognitive tested with six parents facing decisions and five pediatric intensivists. Ten parents facing decisions evaluated the booklet using the Preparation for Decision Making Scale and reported their decisional conflict, which was juxtaposed to the conflict of 21 parents who did not read it, using the Decisional Conflict Scale. Twelve home ventilation program directors evaluated the booklet's clinical sensibility and sensitivity, using a self-designed six-item questionnaire. Data presented using summary statistics. RESULTS: The illustrated booklet (6th-grade reading level) has nine topical sections on chronic respiratory failure and invasive and noninvasive LTV, including the option to forgo LTV. Ten parents who read the booklet rated it as helping "Quite a bit" or more on all items of the Preparation for Decision Making Scale and had seemingly less decisional conflict than 21 parents who did not. Twelve directors rated it highly for clinical sensibility and sensitivity. CONCLUSIONS: The LTV booklet was rigorously developed and favorably evaluated. It offers a resource to improve patient/family knowledge, supplement shared decision-making, and reduce decisional conflict around LTV decisions. Future studies should validate it in other settings and further study its effectiveness.

Therapy at 30 days of life predicts lung function at 6 to 12 months in infants with congenital diaphragmatic hernia.

INTRODUCTION: Congenital diaphragmatic hernia (CDH) is associated with variable degrees of lung hypoplasia. Pulmonary support at 30 days postnatal age was found to be the strongest predictor of inpatient mortality and morbidity among CDH infants and was also associated with higher pulmonary morbidity at 1 and 5 years. It is not known, however, if there is a relationship between the need for medical therapy at 30 days of life and subsequent abnormalities in lung function as reflected in infant pulmonary function test (iPFT) measurements. OBJECTIVE: We hypothesized that CDH infants who require more intensive therapy at 30 days would have more abnormal iPFT values at the time of their first infant pulmonary function study, reflecting the more severe spectrum of lung hypoplasia. METHODS: A single-institution chart review of all CDH survivors who were enrolled in a Pulmonary Hypoplasia Program (PHP) through July 2019, and treated from 2002 to 2019 was performed. All infants were divided into groups based on their need for noninvasive (supplemental oxygen, high flow therapy, noninvasive mechanical ventilation) or invasive (mechanical ventilation, extracorporeal membrane oxygenation) respiratory assistance, bronchodilators, diuretic use, and pulmonary hypertension (PH) therapy (inhaled and/or systemic drugs) at 30 days. Descriptive and statistical analyses were performed between groups comparing subsequent lung function measurements. RESULTS: A total of 382 infants (median gestational age [GA] 38.4 [interquartile range (IQR) = 37.1-39] weeks, 41.8% female, 70.9% Caucasian) with CDH were enrolled in the PHP through July 2019, and 118 infants underwent iPFT. The median age of the first iPFT was 6.6 (IQR = 5.3-11.7) months. Those requiring any pulmonary support at 30 days had a higher functional residual capacity (FRC) (z) (P = .03), residual volume (RV) (z) (P = .008), ratio of RV to total lung capacity (RV/TLC) (z) (P = .0001), and ratio of FRC to TLC (FRC/TLC) (z) (P = .001); a lower forced expiratory volume at 0.5 seconds (FEV0.5) (z) (P = .03) and a lower respiratory system compliance (Crs) (P = .01) than those who did not require any support. Similarly, those requiring diuretics and/or PH therapy at 30 days had higher fractional lung volumes, lower forced expiratory flows and Crs than infants who did not require such support (P < .05). CONCLUSIONS: Infants requiring any pulmonary support, diuretics and/or PH therapy at 30 postnatal days have lower forced expiratory flows and higher fractional lung volumes, suggesting a greater degree of lung hypoplasia. Our study suggests that the continued need for PH, diuretic or pulmonary support therapy at 30 days can be used as additional risk-stratification measurements for evaluation of infants with CDH.

Transition of patients with neuromuscular disease and chronic ventilator-dependent respiratory failure from pediatric to adult pulmonary care.

Improvements in medical care have allowed many children with neuromuscular disease and chronic respiratory failure to survive into adulthood. There are currently no published guidelines to facilitate transition from pediatric to adult respiratory care in this population. The transition process in neuromuscular disease and chronic respiratory failure is uniquely challenging in that the patients are often declining in health and losing independence as they approach adulthood. Barriers to transition include lack of access to adult providers, incompatible health insurance, loss of resources within patients' medical structures, absence of transition preparation, and patient and family insecurity with a new healthcare system. The six core elements and optimal time frame of transition should be applied, with special consideration of the psychosocial aspects associated with neuromuscular disease. Successful transition revolves around information, open communication between young adults and their medical care team, and individualized planning to ensure optimal health and quality of life.

Decisions for Long-Term Ventilation for Children. Perspectives of Family Members.

Rationale: The decision whether to initiate or forgo long-term ventilation for children can be difficult and impactful. However, little has been published on the informational and decisional needs of families facing this decision.Objectives: To assess what families with children with chronic respiratory failure and life-limiting conditions need and want for informed decision-making.Methods: English- and Spanish-speaking parents who were facing (contemporaneous decision makers) or had previously faced (former decision makers) a decision regarding invasive or noninvasive long-term ventilation for their children were recruited using convenience sampling. Patients who were older and cognitively capable also were invited to participate. We performed semistructured interviews using an open-ended interview guide developed de novo to assess parents' decisional needs and experiences. Qualitative data analysis used a thematic approach based on framework analysis, and thematic saturation was a goal.Results: A sample of 44 parents and 2 patients from 43 families was interviewed. All contemporaneous decision makers (n = 28) favored or believed that they would choose long-term ventilation. Fifteen of 16 former decision makers chose long-term ventilation. Thematic saturation was achieved from the perspective of parents who favored or chose long-term ventilation. Four domains were identified: parents' emotional and psychological experiences with decision-making, parents' informational needs, parents' communication and decision support needs, and parents' views on the option not to initiate long-term ventilation. For most parents, making a decision regarding long-term ventilation was stressful, even though they articulated goals and values that could/did guide their decision-making. In general, parents wanted comprehensive information, including what life would be like at home for the child and the family. They wanted their medical providers to be honest, tactful, patient, and supportive. Parents reported that they felt being presented with the option not to initiate was acceptable.Conclusions: In this study, we identified specific informational and decision-making needs regarding long-term ventilation that parents facing decisions feel are important. These data suggest that providers should present families with comprehensive, balanced information on the impact of long-term ventilation and, when the child has a profoundly serious and life-limiting condition, explore the option not to initiate long-term ventilation.