Tracheal surgery for airway anomalies associated with increased mortality in pediatric patients undergoing heart surgery: Society of Thoracic Surgeons Database analysis.

OBJECTIVES: Airway anomalies are common in children with cardiac disease but with an unquantified impact on outcomes. We sought to define the association between airway anomalies and tracheal surgery with cardiac surgery outcomes using the Society of Thoracic Surgery Congenital Heart Surgery Database. METHODS: Index cardiac operations in children aged less than 18 years (January 2010 to September 2018) were identified from the Society of Thoracic Surgery Congenital Heart Surgery Database. Patients were divided on the basis of reported diagnosis of an airway anomaly and subdivided on the basis of tracheal lesion and tracheal surgery. Multivariable analysis evaluated associations between airway disease and outcomes controlling for covariates from the Society of Thoracic Surgery Congenital Heart Surgery Database Mortality Risk Model. RESULTS: Of 198,674 index cardiovascular operations, 6861 (3.4%) were performed in patients with airway anomalies, including 428 patients (0.2%) who also underwent tracheal operations during the same hospitalization. Patients with airway anomalies underwent more complex cardiac operations (45% vs 36% Society of Thoracic Surgeons/European Association for Cardiothoracic Surgery Congenital Heart Surgery Mortality category ≥3 procedures) and had a higher prevalence of preoperative risk factors (73% vs 39%; both P < .001). In multivariable analysis, patients with airway anomalies had increased odds of major morbidity and tracheostomy (P < .001). Operative mortality was also increased in patients with airway anomalies, except those with malacia. Tracheal surgery within the same hospitalization increased the odds of operative mortality (adjusted odds ratio, 3.9; P < .0001), major morbidity (adjusted odds ratio, 3.7; P < .0001), and tracheostomy (adjusted odds ratio, 16.7; P < .0001). CONCLUSIONS: Patients undergoing cardiac surgery and tracheal surgery are at significantly higher risk of morbidity and mortality than patients receiving cardiac surgery alone. Most of those with unoperated airway anomalies have higher morbidity and mortality, which makes it an important preoperative consideration.

Prenatal Imaging to Predict Need for Urgent Perinatal Surgery in Congenital Lung Lesions.

BACKGROUND: Congenital lung malformations (CLMs) have a variable natural history: some patients require urgent perinatal surgical intervention (UPSI) and others remain asymptomatic. These lesions have potential growth until 26-28 wk gestation. CLM volume ratio (CVR) has been shown to predict the risk of hydrops in CLMs. However, no criteria exist to delineate lesions requiring urgent surgical intervention in the perinatal period. Our goal was to determine prenatal diagnostic features that predict the need for UPSI in patients diagnosed with CLM. METHODS: Records and imaging features of all fetuses evaluated by our fetal center between May 2015 and December 2018 were retrospectively reviewed. Data included demographics, fetal ultrasound and magnetic resonance imaging, CVR, surgical treatment, and outcome. Features were analyzed for their ability to predict the need for UPSI. RESULTS: Sixty-four patients were referred for CLM, with 48 patients serially followed. Nine (18.8%) patients were followed nonoperatively, 35 (72.9%) underwent resection, and four (8.3%) were lost to follow-up. Of the patients who underwent resection, 24 (68.5%) were electively resected and 11 were urgently resected. Five (14.3%) patients underwent ex utero intrapartum treatment resection, and six (17.1%) were urgently resected for symptomatic CLM. There were no cases of UPSI with final CVR <1.1. Of the patients with final CVR 1.1-1.7, 43% required urgent resection. CVR ≥1.1 has 100% sensitivity and 87.8% specificity to predict patients requiring UPSI (area under the curve of 0.98). CONCLUSIONS: A final CVR ≥1.1 is highly predictive for UPSI. Patients with a final CVR ≥1.1 should be referred for delivery at centers with pediatric surgeons equipped for potential UPSI for CLM.

Apnea-Hypopnea Event Duration Predicts Mortality in Men and Women in the Sleep Heart Health Study.

RATIONALE: Obstructive sleep apnea is a risk factor for mortality, but its diagnostic metric-the apnea-hypopnea index-is a poor risk predictor. The apnea-hypopnea index does not capture the range of physiological variability within and between patients, such as degree of hypoxemia and sleep fragmentation, that reflect differences in pathophysiological contributions of airway collapsibility, chemoreceptive negative feedback loop gain, and arousal threshold. OBJECTIVES: To test whether respiratory event duration, a heritable sleep apnea trait reflective of arousal threshold, predicts all-cause mortality. METHODS: Mortality risk as a function of event duration was estimated by Cox proportional hazards in the Sleep Heart Health Study, a prospective community-based cohort. Gender-specific hazard ratios were also calculated. MEASUREMENTS AND MAIN RESULTS: Among 5,712 participants, 1,290 deaths occurred over 11 years of follow-up. After adjusting for demographic factors (mean age, 63 yr; 52% female), apnea-hypopnea index (mean, 13.8; SD, 15.0), smoking, and prevalent cardiometabolic disease, individuals with the shortest-duration events had a significant hazard ratio for all-cause mortality of 1.31 (95% confidence interval, 1.11-1.54). This relationship was observed in both men and women and was strongest in those with moderate sleep apnea (hazard ratio, 1.59; 95% confidence interval, 1.11-2.28). CONCLUSIONS: Short respiratory event duration, a marker for low arousal threshold, predicts mortality in men and women. Individuals with shorter respiratory events may be predisposed to increased ventilatory instability and/or have augmented autonomic nervous system responses that increase the likelihood of adverse health outcomes, underscoring the importance of assessing physiological variation in obstructive sleep apnea.

The comorbidities and risk factors in children with congenital airway anomalies: A nationwide population-based study in Taiwan.

The comorbidities and risk factors associated with congenital airway anomalies (CAAs) in children are undecided. This study aimed to investigate the comorbidities commonly associated with CAA and to explore the prognosis and risk factors in CAA children.This nationwide, population-based cohort study was conducted between 2000 and 2011 with children aged 0 to 5 years assigned to either a CAA group (6341 patients) that diagnosed with CAA or an age- and gender-matched control group (25,159 patients) without CAA, using the Taiwan National Health Insurance Research Database (NHIRD). Descriptive, logistic regression, Kaplan-Meier, and Cox regression analyses were used for the investigation.Cleft lip/palate (adjusted odds ratio [aOR], 7.88; 95% confidence interval [CI], 6.49-9.59), chromosome (aOR, 6.85; 95% CI, 5.03-9.34), and congenital neurologic (aOR, 5.52; 95% CI, 4.45-6.87) anomalies were the comorbidities most highly associated with CAA. Of the 31,500 eligible study patients, 636 (399 in the CAA group and 237 in the control group) died during the follow-up period (6.3% vs 0.9%, P < .001). The mortality risk after adjusting for age, gender, and comorbidities elevated significantly among CAA patients (adjusted hazard ratio [aHR], 4.59; 95% CI, 3.85-5.48). The need for tracheostomy (aHR, 2.98; 95% CI, 2.15-4.15), comorbidity with congenital heart disease (CHD) (aHR, 2.52; 95% CI, 2.05-3.10), and chromosome anomaly (aHR, 2.34; 95% CI, 1.70-3.23) were the independent risk factors most greatly related to CAA mortality.This study demonstrated that CAA was most highly associated with the comorbidities as cleft lip/palate, chromosome, and congenital neurologic anomalies. The CAA children had a significantly elevated mortality risk; the need for tracheostomy, CHD, and chromosome anomaly were the most related risk factors of mortality for CAA. Further studies are warranted to clarify the involved mechanisms.

Resection of pediatric lung malformations: National trends in resource utilization & outcomes.

PURPOSE: We sought to determine factors influencing survival and resource utilization in patients undergoing surgical resection of congenital lung malformations (CLM). Additionally, we used propensity score-matched analysis (PSMA) to compare these outcomes for thoracoscopic versus open surgical approaches. METHODS: Kids' Inpatient Database (1997-2009) was used to identify congenital pulmonary airway malformation (CPAM) and pulmonary sequestration (PS) patients undergoing resection. Open and thoracoscopic CPAM resections were compared using PSMA. RESULTS: 1547 cases comprised the cohort. In-hospital survival was 97%. Mortality was higher in small vs. large hospitals, p<0.005. Survival, pneumothorax (PTX), and thoracoscopic procedure rates were higher, while transfusion rates and length of stay (LOS) were lower, in children ≥3 vs. <3months (p<0.001). Multivariate analysis demonstrated longer LOS for older patients and Medicaid patients (all p<0.005). Total charges (TC) were higher for Western U.S., older children, and Medicaid patients (p<0.02). PSMA for thoracoscopy vs. thoracotomy in CPAM patients showed no difference in outcomes. CONCLUSION: CLM resections have high associated survival. Children <3months of age had higher rates of thoracotomy, transfusion, and mortality. Socioeconomic status, age, and region were independent indicators for resource utilization. Extent of resection was an independent prognostic indicator for in-hospital survival. On PSMA, thoracoscopic resection does not affect outcomes.

Prognosis and Risk Factors for Congenital Airway Anomalies in Children with Congenital Heart Disease: A Nationwide Population-Based Study in Taiwan.

BACKGROUND: The mortality risk associated with congenital airway anomalies (CAA) in children with congenital heart disease (CHD) is unclear. This study aimed to investigate the factors associated with CAA, and the associated mortality risk, among children with CHD. METHODS: This nationwide, population-based study evaluated 39,652 children with CHD aged 0-5 years between 2000 and 2011, using the Taiwan National Health Insurance Research Database (NHIRD). We performed descriptive, logistic regression, Kaplan-Meier, and Cox regression analyses of the data. RESULTS: Among the children with CHD, 1,591 (4.0%) had concomitant CAA. Children with CHD had an increased likelihood of CAA if they were boys (odds ratio [OR], 1.48; 95% confidence interval [CI], 1.33-1.64), infants (OR, 5.42; 95%CI, 4.06-7.24), or had a congenital musculoskeletal anomaly (OR, 3.19; 95%CI, 2.67-3.81), and were typically identified 0-3 years after CHD diagnosis (OR, 1.33; 95%CI 1.17-1.51). The mortality risk was increased in children with CHD and CAA (crude hazard ratio [HR], 2.05; 95%CI, 1.77-2.37), even after adjusting for confounders (adjusted HR, 1.76; 95%CI, 1.51-2.04). Mortality risk also changed by age and sex (adjusted HR and 95%CI are quoted): neonates, infants, and toddlers and preschool children, 1.67 (1.40-2.00), 1.93 (1.47-2.55), and 4.77 (1.39-16.44), respectively; and boys and girls, 1.62 (1.32-1.98) and 2.01 (1.61-2.50), respectively. CONCLUSION: The mortality risk is significantly increased among children with CHD and comorbid CAA. Clinicians should actively seek CAA during the follow-up of children with CHD.

Thoracoscopy in the management of congenital lung diseases in infancy.

BACKGROUND: This study aimed to compare the results of thoracoscopic surgery for congenital lung diseases between infants younger than 6 months and those older than 6 months at the time of surgery in terms of operation duration, surgical complications, chest tube duration, and hospital stay. METHODS: The charts of 30 thoracoscopic resections for congenital lung diseases were retrospectively reviewed. This study compared 17 children younger than 6 months (mean, 3.94 months; range, 0.37-5.7 months; group 1) with 13 children older than 6 months (mean, 12.05 months; range, 6.2-24.63 months; group 2) at the time of surgery. The median follow-up period was 9 months (range, 1-41 months). RESULTS: Lobectomy was performed in 27 cases, bilobectomy in 1 case, and nonanatomic excision in 2 cases. The mean operating time for group 1 (176±54 min) was similar to that for group 2 (160±46 min). The difference is not significant. The mean duration of chest tube drainage was similar in the two groups (4.4 days; range, 1-9 days for group 1 vs. 4.1 days; range, 3-8 days for group 2). The complications included 1 major and 10 minor complications, with no statistically significant difference between the two groups. Three surgical procedures in each group were converted. The hospital stay was not statistically different between the two groups (8 days; range, 3-20 days for group 1 vs. 6 days; range, 4-10 days for group 2). CONCLUSIONS: The study findings showed no statistically significant difference between the two groups in terms of operation time, complication rate, conversion rate, or hospital stay. Lobectomy can be safely and successfully performed by thoracoscopy even for children younger than 6 months.

Patient characteristics associated with in-hospital mortality in children following tracheotomy.

OBJECTIVES: To identify children at risk for in-hospital mortality following tracheotomy. DESIGN: Retrospective cohort study. SETTING: 25 746 876 US hospitalisations for children within the Kids' Inpatient Database 1997, 2000, 2003 and 2006. PARTICIPANTS: 18 806 hospitalisations of children ages 0-18 years undergoing tracheotomy, identified from ICD-9-CM tracheotomy procedure codes. MAIN OUTCOME MEASURE: Mortality during the initial hospitalisation when tracheotomy was performed in relation to patient demographic and clinical characteristics (neuromuscular impairment (NI), chronic lung disease, upper airway anomaly, prematurity, congenital heart disease, upper airway infection and trauma) identified with ICD-9-CM codes. RESULTS: Between 1997 and 2006, mortality following tracheotomy ranged from 7.7% to 8.5%. In each year, higher mortality was observed in children undergoing tracheotomy who were aged <1 year compared with children aged 1-4 years (mortality range: 10.2-13.1% vs 1.1-4.2%); in children with congenital heart disease, compared with children without congenital heart disease (13.1-18.7% vs 6.2-7.1%) and in children with prematurity, compared with children who were not premature (13.0-19.4% vs 6.8-7.3%). Lower mortality was observed in children with an upper airway anomaly compared with children without an upper airway anomaly (1.5-5.1% vs 9.1-10.3%). In 2006, the highest mortality (40.0%) was observed in premature children with NI and congenital heart disease, who did not have an upper airway anomaly. CONCLUSIONS: Congenital heart disease, prematurity, the absence of an upper airway anomaly and age <1 year were characteristics associated with higher mortality in children following tracheotomy. These findings may assist provider communication with children and families regarding early prognosis following tracheotomy.

Predictors of clinical outcomes and hospital resource use of children after tracheotomy.

OBJECTIVES: The objectives are to describe health outcomes and hospital resource use of children after tracheotomy and identify patient characteristics that correlate with outcomes and hospital resource use. PATIENTS AND METHODS: A retrospective analysis of 917 children aged 0 to 18 years undergoing tracheotomy from 36 children's hospitals in 2002 with follow-up through 2007. Children were identified from ICD-9-CM tracheotomy procedure codes. Comorbid conditions (neurologic impairment [NI], chronic lung disease, upper airway anomaly, prematurity, and trauma) were identified with ICD-9-CM diagnostic codes. Patient characteristics were compared with in-hospital mortality, decannulation, and hospital resource use by using generalized estimating equations. RESULTS: Forty-eight percent of children were

A contemporary evaluation of surgical outcome in neonates and infants undergoing lung resection.

BACKGROUND/PURPOSE: The timing and need of resection of asymptomatic congenital lung lesions are controversial. The morbidity of such surgery needs to be considered in the decision analysis. We analyzed the contemporary outcome of infants and neonates undergoing lung resection. METHODS: With institutional review board approval, all patients 12 months or younger undergoing lung resection between 1995 and 2004 in 2 hospitals were reviewed. Demographic data, indications for surgery, operative procedure, complications, use of regional anesthesia, length of stay (LOS), and follow-up were assessed. RESULTS: Forty-five patients (28 male, 17 female) with a median age of 4 months (2 days-12 months) were evaluated. Congenital lesions (42) were the most frequent indication for surgery. Twenty-two (48.9%) patients had cardiorespiratory symptoms or infection preoperatively. Lobectomy was the most common operation (40/45). Three patients had intraoperative difficulty (bleeding, hypotension, desaturation). Significant postoperative complications occurred in 7 patients: prolonged air leak or chest tube drainage (4), anemia or bleeding (2), respiratory distress requiring reintubation (1). Fewer complications occurred in asymptomatic vs symptomatic patients (1/23 vs 6/22). Of 12 patients, 7 (58%) requiring 24 hours of ventilation or longer were 3 months or younger. Increasing age did significantly influence the chance of successful extubation (P = .01; odds ratio, 1.5; 95% confidence interval, 1.0-2.0), as did the use of epidural anesthesia (P < .001). Median LOS was 6 days (2-89 days). Asymptomatic patients had shorter LOS (median, 4 days; range, 2-20 days; P = .024) vs symptomatic patients (median, 8 days; range, 4-89 days). The only death occurred from underlying heart disease. Mean follow-up at 35 months (12-132 months) revealed no subjective reduction in cardiopulmonary function. CONCLUSIONS: Lung resection is safe and well tolerated in infancy. Surgery should be scheduled before the development of symptoms but likely after 3 months of age to improve the chances of postoperative extubation. The use of regional anesthesia may facilitate this.

Respiratory systems abnormalities and clinical milestones for patients with amyotrophic lateral sclerosis with emphasis upon survival.

Respiratory system complications and abnormalities are common in patients with amyotrophic lateral sclerosis (ALS) and respiratory failure remains the most common cause of death. Extensive epidemiological longitudinal data have documented the extent, magnitude, and clinical course of these abnormalities, but few studies have provided objective information that can have prognostic significance for individual patients. In this study, the reported data represent results from a retrospective review of the medical records of 153 patients with ALS cared for at a single institution (The Penn State Milton S. Hershey Medical Center) over a 50-month period. Medical information in relation to respiratory system abnormalities and complications including pulmonary function measurements was extracted for data analyses. The intent of this review of longitudinal data from a relatively large cohort of patients with ALS was to identify clinically relevant easily-identifiable objective information and clinical milestones that could have potential prognostic significance when applied to individual patients. Demographic data including gender, survival outcome, respiratory symptoms, age of disease onset, and age at death were similar to previously published epidemiological studies: mean age at ALS disease onset was 58.9+/-12.7 years, and mean age at death was 66.7+/-10.8 years. For 151 patients with available data, the incidence of study defined respiratory complications included infectious pneumonia 13 (9%), venothromboembolism 9 (6%), and tracheostomy and mechanical ventilation 6 (4%). For 139 patients with serial measurements of forced vital capacity (FVC), median values for calculated rate of decline in FVC was 97 ml/30 days (2.4% predicted/30 days); 25% of patients had FVC rates of decline less than 52 ml/30 days (1.4% predicted/30 days) and 25% had rates of decline greater than 170 ml/30 days (4.4% predicted/30 days). Stratifying patients into two distinct clinical subgroups based upon rates of decline in FVC less than or greater than the median value of 97 ml/30 days identified an apparent two-fold increase in survival duration for ALS patients with slower rates of pulmonary physiology deterioration when referenced to either date of dyspnea onset or time from bi-level positive airway pressure (BiPAP) initiation (2.0+/-1.4 vs. 1.0+/-0.8 years; 1.9+/-1.5 vs. 1.0+/-0.9 years, respectively). We concluded that the correlation between clinically defined milestones, most importantly onset of dyspnea, and the calculated rate of decline in FVC represent obtainable and objective measurements that predict the natural course of respiratory muscle dysfunction in patients with ALS and provide important prognostic information in relation to individual patient survival duration.

Congenital diaphragmatic hernia: a retinoid-signaling pathway disruption during lung development?

Congenital diaphragmatic hernia (CDH) usually occurs sporadically. The prognosis remains poor, with a 50% perinatal mortality rate. Most deaths result from hypoxemia due to lung hypoplasia and abnormal development of pulmonary vasculature that results in persistent pulmonary hypertension. Our current understanding of the pathogenesis of CDH is based on an assumption linking herniation of abdominal viscera into the thorax with compression of the developing lung. Pulmonary hypoplasia, however, can also result from reduced distension of the developing lung secondary to impaired fetal breathing movements. Moreover, a nitrofen-induced CDH model shows that lung hypoplasia precedes the diaphragmatic defect, leading to a "dual-hit hypothesis." Recent data reveal the role of a retinoid-signaling pathway disruption in the pathogenesis of CDH. We describe the clinical and epidemiological aspects of human CDH, the metabolic and molecular aspects of the retinoid-signaling pathway, and the implications of retinoids in the development of the diaphragm and the lung. Finally, we highlight the existing links between CDH and disruption of the retinoid-signaling pathway, which may suggest an eventual use of retinoids in the treatment of CDH.