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Cardiac dysfunction is a key determinant of outcome in congenital diaphragmatic hernia (CDH). Pro-b-type natriuretic peptide (proBNP) is used as a prognosticator in heart failure and cardiomyopathy. We hypothesized that proBNP levels would be associated with ventricular dysfunction and high-risk disease in CDH.Patients in the CDH Study Group (CDHSG) from 2015-2019 with at least one proBNP value were included. Ventricular function was determined using echocardiograms from the first 48 h of life.A total of 2,337 patients were identified, and 212 (9%) had at least one proBNP value. Of those, 3 (1.5%) patients had CDHSG stage A defects, 58 (29.6%) B, 111 (56.6%) C, and 24 (12.2%) D. Patients with high-risk defects (Stage C/D) had higher proBNP compared with low-risk defects (Stage A/B) (14,281 vs. 5,025, p = 0.007). ProBNP was significantly elevated in patients who died (median 14,100, IQR 4,377-22,900 vs 4,911, IQR 1,883-9,810) (p0.001). Ventricular dysfunction was associated with higher proBNP than normal ventricular function (8,379 vs. 4,778, p = 0.005). No proBNP value was both sensitive and specific for ventricular dysfunction (AUC=0.61).Among CDH patients, elevated proBNP was associated with high-risk defects, ventricular dysfunction, and mortality. ProBNP shows promise as a biomarker in CDH-associated cardiac dysfunction.

The purpose of this study was to analyze the clinical characteristics and outcomes of low birthweight (LBW) infants with congenital diaphragmatic hernia (CDH) compared to normal birthweight (NBW) infants with CDH. We hypothesized that LBW was associated with increased mortality, decreased extracorporeal life support (ECLS) utilization, and increased pulmonary morbidity in CDH patients. Patients in the CDH Study Group from 2007 to 2018 were included. LBW was defined as p = .03). LBW infants had longer durations of mechanical ventilation and were more likely to require supplemental oxygen at 30 days and at the time of discharge. LBW is a risk factor for mortality and pulmonary morbidity in CDH. Prolonged oxygen requirement and increased length of stay are important considerations when managing this population.

Objectives Given significant focus on improving survival for "high-risk" congenital diaphragmatic hernia, there is the potential to overlook the need to identify risk factors for suboptimal outcomes in "low-risk" congenital diaphragmatic hernia cases. We hypothesized that early cardiac dysfunction or severe pulmonary hypertension were predictors of adverse outcomes in this "low-risk" congenital diaphragmatic hernia population. Design This is a retrospective cohort study using data from the Congenital Diaphragmatic Hernia Study Group registry. "Low-risk" congenital diaphragmatic hernia was defined as Congenital Diaphragmatic Hernia Study Group defect size A/B without structural cardiac and chromosomal anomalies. Examined risk factors included left ventricular dysfunction, right ventricular dysfunction, and severe pulmonary hypertension on the first postnatal echocardiogram. The primary outcome was composite adverse events, defined as either death, extracorporeal membrane oxygenation utilization, oxygen requirement on day 30 of life, or hospitalization greater than or equal to 8 weeks. Multivariable adjustment was performed with logistic regression and inverse probability weighting. Setting Neonatal index hospitalization for congenital diaphragmatic hernia. Patients "Low-risk" congenital diaphragmatic hernia infants born between January 2015 and December 2018. Interventions First postnatal echocardiogram performed within 24 hours from birth. Measurements and main results Seven-hundred seventy-eight patients were identified as "low-risk" congenital diaphragmatic hernia. Left ventricular dysfunction, right ventricular dysfunction, and severe pulmonary hypertension were present in 10.8%, 20.5%, and 57.5%, respectively. The primary outcome occurred in 21.3%. Death occurred in 3.0% and 9.1% used extracorporeal membrane oxygenation. On unadjusted analysis, all three risk factors were associated with the primary outcome. On all multivariable adjustment methods, left ventricular dysfunction and severe pulmonary hypertension remained significant predictors of adverse outcomes while right ventricular dysfunction no longer demonstrated any effect. Conclusions Early left ventricular dysfunction and severe pulmonary hypertension are independent predictors of adverse outcomes among "low-risk" congenital diaphragmatic hernia infants. Early recognition may lead to interventions that can improve outcome in this at-risk cohort.

Rationale: Congenital diaphragmatic hernia (CDH) is an anomaly with a high morbidity and mortality. Cardiac dysfunction may be an important and underrecognized contributor to CDH pathophysiology an...

To determine the association between prenatal ultrasound (US) and magnetic resonance imaging (MRI) characteristics in right congenital diaphragmatic hernia (RCDH) with postnatal outcome.CDH Study Group data were reviewed for all RCDH infants (n = 156) born between 2015 and 2019. Prenatal US and MRI lung size measurements were correlated with survival, extracorporeal life support (ECLS), and defect size.Overall survival was 64.1%. ECLS was required in 40.4%. US and MRI-based prenatal assessment of pulmonary hypoplasia does not predict survival. Prenatal measurement of lung size using either US or MRI correlates with ECLS use. Only MRI-based measures of lung size are associated with defect size.Image-based prenatal predictors of survival, ECLS, and defect size are of limited value in RCDH. Extrapolation of prenatal survival and morbidity indicators from left to right-sided CDH is not appropriate. There is an urgent need to develop RCDH prenatal prediction models.

To evaluate the association between prenatal imaging predictors of patients with left-sided congenital diaphragmatic hernia (LCDH) and postnatal outcomes.CDH study group data were reviewed for LCDH infants born 2015-2019. Prenatal ultrasound (US) and magnetic resonance imaging (MRI) data were collected and correlated with postnatal information including CDHSG defect size (A through D or non-repair (NR)).In total, 929 LCDH patients were included. Both US and MRI imaging predictors correlated with postnatal survival (72.2%) and ECLS use (29.6%). Logistic regression models confirmed increased survival and decreased ECLS use with larger values for all predictors. Importantly, all prenatal values evaluated showed no significant difference between defect size D and NR patients.This is the largest cohort of LCDH patients and demonstrates that prenatal imaging factors correlate with postnatal outcomes and confirms that patients in the non-repair group are prenatally similar to type D defects.

There is a known association between Cornelia de Lange syndrome (CdLS) and congenital diaphragmatic hernia (CDH), with CDH being the cause of death in 5%-20% of CdLS cases. We aimed to identify and describe patients with CDLS and CDH. We hypothesized that CdLS would be associated with high-risk CDH and poor outcomes.CDH Study Group patients from 1995 to 2019 were included. Those with CdLS were reviewed retrospectively. Rates of repair and outcomes were compared between patients with and without CdLS.We identified 9,251 CDH patients. Of those, 21 had confirmed CdLS. CdLS patients had a lower birth weight (2.2±0.57 kg) than non-CdLS patients (2.9±0.64 kg) (p0.001). 5-min Apgar scores were lower in CdLS patients (6, 4-7) than non-CdLS patients (7, 5-8) (p=0.014). Only 33% of CdLS patients underwent diaphragmatic repair compared to 84.2% of non-CdLS patients (p0.001). Mortality was 76% for CdLS patients compared with 29% for non-CdLS patients (p0.001). Of the 7 CdLS patients who underwent repair, 5 survived to hospital discharge.Infants with CdLS and CDH have a poor prognosis. However, CdLS patients who undergo repair can survive to discharge; therefore, the concomitant diagnosis of CdLS and CDH is not necessarily a contraindication to repair. Early recognition of these anomalies can assist with counseling and prognostication.Retrospective comparative study LEVEL OF EVIDENCE: III.

OBJECTIVE To determine if risk-adjusted survival of patients with congenital diaphragmatic hernia (CDH) has improved over the last 25 years within centers that are long-term, consistent participants in the CDH Study Group (CDHSG). SUMMARY BACKGROUND DATA The CDHSG is a multicenter collaboration focused on evaluation of infants with CDH. Despite advances in pediatric surgical and intensive care, CDH mortality has appeared to plateau. Herein, we studied CDH mortality rates amongst long-term contributors to the CDHSG. METHODS We divided registry data into five-year intervals, with Era 1 (E1) beginning in 1995, and analyzed multiple variables (operative strategy, defect size, and mortality) to assess evolution of disease characteristics and severity over time. For mortality analyses, patients were risk stratified using a validated prediction score based on 5-minute Apgar (Apgar5) and birth weight. A risk-adjusted, observed to expected (O:E) mortality model was created using E1 as a reference. RESULTS 5,203 patients from 23 centers with ≥22 years of participation were included. Birth weight, Apgar5, diaphragmatic agenesis, and repair rate were unchanged over time (all p > 0.05). In E5 compared to E1, minimally invasive and patch repair were more prevalent, and timing of diaphragmatic repair was later (all p < 0.01). Overall mortality decreased over time: E1 (30.7%), E2 (30.3%), E3 (28.7%), E4 (26.0%), E5 (25.8%) (p = 0.03). Risk-adjusted mortality showed a significant improvement in E5 compared to E1 (OR 0.78, 95% CI 0.62-0.98; p = 0.03). O:E mortality improved over time, with the greatest improvement in E5. CONCLUSIONS Risk-adjusted and observed-to-expected CDH mortality have improved over time.

Aim To compare outcomes between prenatally and postnatally diagnosed CDH in a large multicenter database of prospectively collected data and evaluate factors associated with poorer outcome for prenatally diagnosed CDH. Material and Methods We used information from the multicenter, multinational CDH Study Group database on patients born between 2007 and 2015. We compared differences between prenatally and postnatally diagnosed CDH with respect to survival, side, size, ECMO needs, associated major cardiac malformations and liver position. Results 3746 cases of CDH were entered in the registry between 2007 and 2015, with an overall survival of 71%. Of those, 68% had a prenatal diagnosis. Survival rates were significantly better in the postnatally diagnosed group, 83 vs 65%. There was a higher proportion of bigger defect sizes, C and D, in the prenatally diagnosed group, but the survival rates were similar when patients were stratified by defect size. The rate of ECMO utilization was higher overall in the prenatally diagnosed group, 33 vs 22%, but it was similar within similar defect sizes. Right-sided defects are more commonly missed at prenatal screening than left-sided CDH, 53 vs 35% (p Conclusions Prenatally diagnosed CDH is associated with larger defect sizes compared to those with a postnatal diagnosis, and consequently have higher morbidity and mortality. Right-sided CDH are more often missed at prenatal ultrasound. The increasing rate of prenatal detection requires a clear understanding of accurate risk stratification, in order to counsel families and to provide appropriate perinatal management. Level of Evidence I for a Prognosis Study – This is a high-quality, prospective cohort study with 99% of patients followed to the study end point (death or discharge).

To evaluate the association between hypercarbia in the first 24 h of life and clinical outcomes in infants with congenital diaphragmatic hernia (CDH). Retrospective review of patients entered into the CDHSG registry between 2007–2014. Half of the identified patients were analyzed to identify the PaCO2 value most predictive of mortality. Prediction models for outcomes of death, ECMO, and respiratory support at 30 days of life (DOL) were developed using PaCO2. Remaining half of data was used for validation of study findings. 1878 and 1875 patients were analyzed in the testing and validation groups. Lowest PaCO2≥60 mmHg in the first DOL is highly predictive of death prior to discharge. Prediction models including this variable demonstrate good discrimination for outcomes of death, ECMO, and respiratory support (AUC 0.8808, 0.8279, 0.8065). Lowest PaCO2 in the first DOL is an independent risk factor of mortality and morbidity in CDH.

OBJECTIVE To measure the survival among comparable neonates with CDH supported with and without ECLS. SUMMARY OF BACKGROUND DATA Despite widespread use in the management of newborns with CDH, ECLS has not been consistently associated with improved survival. METHODS A retrospective cohort study was performed using ECLS-eligible CDH Study Group registry patients born between 2007 and 2019. The primary outcome was in-hospital mortality. Neonates who did and did not receive ECLS were matched based on variables affecting risk for the primary outcome. Iterative propensity score-matched, survival (Cox regression and Kaplan-Meier), and center effects analyses were performed to examine the association of ECLS use and mortality. RESULTS Of 5855 ECLS-eligible CDH patients, 1701 (29.1%) received ECLS. "High-risk" patients were best defined as those with a lowest achievable first-day arterial partial pressure of CO2 of ≥60 mm Hg. After propensity score matching, mortality was higher with ECLS (47.8% vs 21.8%, odds ratio 3.3, 95% confidence interval 2.7-4.0, hazard ratio 2.3, P < 0.0001). For the subgroup of high-risk patients, there was lower mortality observed with ECLS (64.2% vs 84.4%, odds ratio 0.33, 95% confidence interval 0.17-0.65, hazard ratio 0.33, P = 0.001). This survival advantage was persistent using multiple matching approaches. However, this ECLS survival advantage was found to occur primarily at high CDH volume centers that offer frequent ECLS for the high-risk subgroup. CONCLUSIONS Use of ECLS is associated with excess mortality for low- and intermediate-risk neonates with CDH. It is associated with a significant survival advantage among high-risk infants, and this advantage is strongly influenced by center CDH volume and ECLS experience.

Introduction: Pulmonary hypertension (PH) is the major pathophysiologic consequence of congenital diaphragmatic hernia (CDH). We aimed to evaluate the association between early CDH-associated PH (CDH-PH) and inpatient outcomes. Methods: The CDH Study Group registry was queried for infants born 2015–2019 with echocardiograms before 48h of life. PH was categorized using echocardiographic findings: none, mild (right ventricular systolic pressure Results: Of 1,472 patients, 86.5% had CDH-PH: 13.9% mild (n = 193), 44.4% moderate (n = 631), and 33.2% severe (n = 468). On adjusted analysis, the primary outcome of mortality or oxygen support at 30 days occurred more frequently in infants with moderate (incidence rate ratio [IRR] 1.8, 95% confidence interval [CI], 1.2–2.6) and severe CDH-PH (IRR 2.0, 95% CI, 1.3–2.9). Extracorporeal life support (ECLS) utilization was associated only with severe CDH-PH after adjustment (IRR 1.8, 95% CI, 1.0–3.3). Discussion/Conclusion: Early, postnatal CDH-PH is independently associated with increased risk for mortality or oxygen support at 30 days and utilization of ECLS. Early echocardiogram is a valuable prognostic tool for early, inpatient outcomes in neonates with CDH.

Background The Severe Pulmonary Hypoplasia and Evaluation for Resuscitative Efforts (SPHERE) protocol was developed to attempt to identify CDH patients with likely lethal pulmonary hypoplasia. We present our experience with this protocol and utilize the CDH Registry to critically assess the protocol. Methods SPHERE patients identified based on prenatal imaging (10/2009–1/2018) were offered ECMO if meeting postnatal physiologic criteria, while others received comfort measures. Within the CDH Registry, patients with suspected severe CDH were identified and separated into “passed” (lowest pCO2 ≤ 100) versus “failed” (lowest pCO2 > 100) groups. Results Of 23 SPHERE patients, 57% (13/23) passed criteria for ECMO and survival was 46% (6/13) in that cohort. Of 4912 patients in the CDH Registry, 265 met criteria. There was no difference in survival rates between those that “passed” (122/227; 54%) versus “failed” (18/38; 47%). However, the latter had longer ECMO runs and more required ventilator/ECMO support at 30 days. Amongst survivors, the “failed” group had longer hospital stays and more frequently required tube feeds at discharge. Conclusions The SPHERE protocol did not predict mortality in the CDH Registry. However, our data suggest resource utilization is significant when unable to reach pCO2 ≤ 100 despite resuscitation. Morbidity remains high in this group. Level of evidence Level III Annotation of changes Institutional Review Board Approval at University of Michigan (HUM00031524 and HUM00044010) Type of Study Retrospective Review

Objective: The objectives of this study were (i) to evaluate infants with congenital diaphragmatic hernia (CDH) that do not undergo repair, (ii) to identify nonrepair rate by institution, and (iii) to compare institutional outcomes based on nonrepair rate. Background: Approximately 20% of infants with CDH go unrepaired and the threshold to offer surgical repair is variable. Methods: Data were abstracted from a multicenter, prospectively collected database. Standard clinical variables, including repair (or nonrepair), and outcome were analyzed. Institutions were grouped based on volume and rate of nonrepair. Preoperative mortality predictors were identified using logistic regression, expected mortality for each center was calculated, and observed /expected (O/E) ratios were computed for center groups and compared by Kruskal-Wallis ANOVA. Results: A total of 3965 infants with CDH were identified and 691 infants (17.5%) were not repaired. Nonrepaired patients had lower Apgar scores (P < 0.05) and increased incidence of anomalies (P < 0.0001). Low-volume centers ("Lo", n=44 total, < 10 CDH pts/yr) and high-volume centers ("Hi", n = 21) had median nonrepair rates of 19.8% (range 0%-66.7%) and 16.7% (5.1%-38.5%), respectively. High-volume centers were further dichotomized by rate of nonrepair (HiLo = 5.1-16.7% and HiHi = 17.6-38.5%), leaving 3 groups: HiLo, HiHi, and Lo. Predictors of mortality were lower birth weight, lower Apgar scores, prenatal diagnosis, and presence of congenital anomalies. O/E ratios for mortality in the HiLo, HiHi, and Lo groups were 0.81, 0.94, and 1.21, respectively (P < 0.0001). For every 100 CDH patients, HiLo centers have 2.73 (2.4-3.1, 95% confidence interval) survivors beyond expectation. Conclusions: There are significant differences between repaired and nonrepaired CDH infants and significant center variation in rate of nonrepair exists. Aggressive surgical management, leading to a low rate of nonrepair, is associated with improved risk-adjusted mortality.

OBJECTIVE To determine the optimal timing of congenital diaphragmatic hernia (CDH) repair after extracorporeal membrane oxygenation (ECMO) cannulation. SUMMARY BACKGROUND DATA The timing of CDH repair after ECMO cannulation remains a controversial topic due to studies with low power or strong selection bias. METHODS This is a 2-aim retrospective cohort study based on the CDH Study Group registry for the period of 2007-2017. Aim 1-Compare On versus After ECMO repair. Aim 2-Compare Early versus Late repair on ECMO. In order to minimize selection bias and account for non-repairs, subjects in each aim were stratified into study groups based on their treatment center's characteristics. In each aim, the study groups were matched based on propensity score (PS). The main outcomes included mortality rate and incidence of non-repair. RESULTS In aim 1, 136 patients remained in each group after PS matching. Compared to the After ECMO group, patients in the On ECMO group demonstrated a lower mortality rate, hazard ratio (HR) 0.54 (0.38, 0.77) (P < 0.001), and lower incidence of non-repair, 5.9% versus 33.8% (P < 0.001). In aim 2, 77 patients remained in each group after PS matching. Compared to the Late group, Early repair of CDH on ECMO was associated with a lower mortality rate, HR 0.51 (0.33, 0.77) (P = 0.002), and lower incidence of non-repair, 9.1% versus 44.2% (P < 0.001). CONCLUSIONS The approach of early repair after ECMO cannulation is associated with improved survival compared to delayed surgical correction.

Background Standardized care may improve outcomes in many diseases including congenital diaphragmatic hernia (CDH). Our study assesses the variability of CDH clinical practice guidelines (CPG) among North American centers. Methods North American member institutions of the CDH Study Group and the Pediatric Surgical Research Collaborative were solicited to submit their CDH CPG. Elements from each CPG were collected and classified according to therapeutic purpose. Elements were assigned to umbrella topics of prenatal assessment, delivery plus initial resuscitation, ventilatory and cardiovascular management, therapeutic targets, analgesia, and criteria for transitions in care. Descriptive analyses were performed to characterize the scope and variability of CPGs. Results Sixty-eight centers provided 40 responses (59%). Of these, 29 (73%) had a CDH CPG, of which 27 were obtained for review. All CPGs had a primary focus of preoperative care. Conventional ventilation was the first-line strategy in all CPGs. Ninety-three percent reported a peak inspiratory pressure limit (mean: 25.2 ± 2 cm H2O). Target oxygenation and ventilatory variables had low coefficients of variation. Two-thirds of CPGs discussed echocardiography, with indications for inhaled nitric oxide, sildenafil, and prostaglandins detailed in 81%, 30%, and 22% of CPGs, respectively. Extracorporeal life support and operative indications were specified in 93% and 59%, respectively, although specific targets for each were highly variable. Conclusions This synthesis of North American CDH CPGs identifies areas of both alignment and variability and provides objective data about individual institutional guidelines in CDH care. These data may inform the development of a consensus-based, multi-institutional approach to standardized CDH management in North America.

Background Management of CDH is highly variable from center to center, as are patient outcomes. The purpose of this study was to examine risk-stratified survival and extracorporeal membrane oxygenation (ECMO) rates at a single center, and to determine whether adverse outcomes are related to patient characteristics or management. Methods A retrospective single-center review of CDH patients was performed, and outcomes compared to those reported by the CDH Study Group (CDHSG) registry. Patient demographics, disparities, and clinical characteristics were examined to identify unique features of the cohort. A model derived using the registry that estimates probability of ECMO use or death in CDH newborns was used to risk-stratify patients and assess mortality rates. Observed over expected (O/E) ECMO use rates were calculated to measure whether “excess” or “appropriate” ECMO use was occurring. Results There were 81 CDH patients treated between 2004–2017, and 5034 in the CDHSG registry. Mortality in ECMO-treated patients was higher than the registry. Socioeconomic variables were not significantly associated with outcomes. The strongest predictors of mortality were ECMO use and early blood gas variables. The risk model accurately predicted ECMO use with a c-statistic of 0.79. Compared with the registry, the disparity in mortality rates was greatest for moderate-risk patients. O/E ECMO use was highest in low and moderate-risk patients. Conclusions ECMO use is a more consistent predictor of mortality than CDH severity at a single center, and there is relative overuse of ECMO in lower-risk patients. Risk stratification allows for more accurate institutional assessment of mortality and ECMO use, and other centers could consider such an adjusted analysis to identify opportunities for outcomes improvement. Level of Evidence III.

Purpose “Early on-ECMO” repair of CDH entails repair within 48–72 h of cannulation in an effort to optimize pulmonary physiology, shorten ECMO duration, and, ultimately, improve survival. This study evaluated the effect of early on-ECMO repair as compared to leaving patients unrepaired during ECMO. Methods The CDH Study Group database was queried for CDH patients requiring ECMO who either underwent repair within the first 72 h after cannulation or remained unrepaired on ECMO. Primary outcomes were survival to decannulation and ECMO duration. Results A total of 248 patients underwent early repair and 922 remained unrepaired on ECMO. The early repair group had increased risk factors for poor outcomes, including higher odds of cardiac defects and thoracic liver location, and lower odds of hernia sac presence. Nonetheless, ECMO survival for the early repair group was 87.1% compared to 78.4% in the unrepaired group (p = 0.002). However, the early repair group had a longer median ECMO duration than the unrepaired group (240.6 vs 196.8 h, p = 0.001). Conclusion While early ECMO repair does not shorten ECMO duration, it results in increased survival to decannulation as compared to those unrepaired on ECMO. This suggests that there may be a physiologic benefit leading to increased ECMO survival in a subset of patients undergoing on-ECMO repair over those designated to undergo post-ECMO repair. Level of evidence Level III.

Optimal timing of congenital diaphragmatic hernia (CDH) repair in patients requiring extracorporeal membrane oxygenation (ECMO) remains controversial. The “late ECMO repair” is an approach where the patient, once deemed stable for decannulation, is repaired while still on ECMO to enable expeditious return to ECMO if surgery induces instability. The goal of this study was to investigate the potential benefit of this approach by evaluating the rate of return to ECMO after repair. The CDH Study Group database was used to analyze CDH patients requiring ECMO support. The primary outcome was return to ECMO within 72 h of CDH repair among those repaired following ECMO decannulation (“post-ECMO” patients). Secondary outcomes were death within 72 h of repair and cumulative death and return to ECMO rate. A total of 668 patients were repaired post-ECMO decannulation. Six patients (0.9%) in the post-ECMO group required return to ECMO within 72 h of surgery and a total of 19 (2.8%) died or returned to ECMO within 72 h of surgery. The rate of return to ECMO and death following CDH repair is extremely low and does not justify the risks inherent to “on-ECMO” repair. Patients stable to come off ECMO should undergo repair after decannulation.

Right-sided congenital diaphragmatic hernias (CDH) and bigger defect sizes have been associated with poorer outcomes.The aim of this study was to evaluate right- and left-sided CDH in terms of size, survival, associated anomalies, and morbidity.We used information from a multicenter, multinational database including patients with CDH born between 2007 and 2015. All infants with data on defect side were included for this analysis. We compared differences in outcomes between right- and left-sided CDH. Further analysis on the association between side, size of the defect, and outcome was performed.A total of 3754 cases of CDH were entered in the registry between January 2007 and September 2015, with an overall survival of 71%. Of those, 598 (16%) were right-sided and 3156 left-sided, with a survival rate of 67% and 72%, respectively. Right-sided CDH had a larger proportion of C and D defects (p0.001 and 0.04, respectively). Survival rates for the same size defect were similar, independent of the side of the defect. Multivariable logistic regression analysis with survival as dependent variable identified a significant correlation with defect size, but not side.The higher proportion of large defects (CD) in right-sided CDH, not the side itself, accounts for the reported poorer survival in right-sided CDH.Level I for a prognosis study - This is a high-quality, prospective cohort study with 99% of patients followed to the study end point (death or discharge).

Objective To report treatment strategies’ evolution and its impact on congenital diaphragmatic hernia (CDH) outcome. Design Registry-based cohort study using the CDH Study Group database, 1995–2013. Setting International multicenter database. Patients CDH patients entered into the registry. Late presenters or patients with very incomplete data were excluded. Patients were divided into three Eras (1995–2000; 2001–2006; 2007–2013). Main outcome measures Treatment strategies and outcomes. One-way ANOVA, X2 test, and X2 test for trend were used. A Sydak-adjusted p

A means for early postnatal stratification of ECMO risk in CDH newborns could be used to comparatively assess the utilization and outcomes of ECMO use between centers. While multiple CDH mortality risk calculators are available, no validated tool exists specifically for prediction of ECMO use. The purpose of this study was to derive and validate an ECMO risk stratification model.The study population was obtained from CDH Study Group registry for the period between 2007 and 2016. Only centers offering ECMO were included. The cohort was restricted to ECMO candidates and then divided into derivation and validation sets. Using all relevant perinatal predictors in the registry, univariate analysis was performed for the composite outcome of ECMO use or death without ECMO use. The model was derived using the derivation cohort with multivariable logistic regression and automatic stepwise forward selection (P 0.05 for qualifying variables), and a c-statistic was obtained. The model was then tested on the validation cohort. Sample reuse validation and bootstrap validation were performed. The validated model was then tested for accuracy on CDH subgroups.There were 1992 patients in the derivation cohort. Four significant variables were identified in the final ECMO risk model: 1-min and 5-min Apgar scores and highest and lowest post-ductal partial pressure of CO2 during the first 24 h of life. The model c-statistic was 0.824 which was confirmed with cross-validation and bootstrap optimism correction. The validation cohort c-statistic was 0.823 (N = 993). The model had good discrimination for left and right CDH, inborn and outborn patients, patients born before and after 2011, and high and low volume centers. The model performed significantly better for postnatally diagnosed patients.This study represents proof-of-concept that a risk model can accurately estimate the probability of ECMO use in CDH newborns. This stratification could assist centers as a metric for assessment of ECMO usage and outcomes. Refinement and prospective validation of this model should be carried out prior to clinical application.3.

Among congenital malformations, congenital diaphragmatic hernia (CDH) is distinguished by its relatively low occurrence rate, need for resource intensive, integrated multidisciplinary care, and widespread variation in practice and outcome. Although randomized controlled trials (RCTs) are considered the gold standard for generating evidence, they are poorly suited to the study of a condition like CDH due to challenges in illness severity adjustment, unpredictability in clinical course and the impact limitations of studying a single intervention at a time. An alternative to RCTs for comparative effectiveness research for CDH is the patient registry, which aggregates multi-institutional condition-specific patient level data into a large CDH-specific database for the dual purposes of collaborative research and quality improvement across participating sites. This article discusses patient registries from the perspective of structure, data collection and management, and privacy protection that guide the use of registry data to support collaborative, multidisciplinary research. Two CDH-specific registries are described as illustrative examples of the "value proposition" of registries in improving the evidence basis for best practices for CDH.

Background The purpose of this study was to identify patient and treatment characteristics associated with early (in hospital) hernia recurrence after congenital diaphragmatic hernia (CDH) repair. Methods Data from the Congenital Diaphragmatic Hernia Study Group registry were queried from 2007 to 2015. Recurrence of the diaphragmatic hernia after initial repair and prior to death or discharge was determined at the time of reoperation. Minimally invasive surgery (MIS) approaches included laparoscopy or thoracoscopy, and open approaches consisted of laparotomy or thoracotomy. Multivariate regression analysis was performed. Results Of 3984 patients, 3332 (84%) underwent CDH repair. 76 (2.3%) patients had an early recurrence. The rate of recurrence was less variable over time for patients undergoing laparotomy vs thoracoscopy (range: 1.1–3.7% vs 1.7–8.9% annually). Timing of repair, whether performed after, during, or before ECMO did not significantly alter recurrence rates (0% vs 4.2% vs 3.0%, p =0.116). Larger defect size (C: OR 4.3, 95% CI 1.2–15.4; D: OR 7.1, 95% CI 1.7–29.1) and an MIS approach (OR 3.2, 95% CI 1.7–6.0) were the only independent predictors of recurrence. Conclusion Larger defect size and an MIS approach were associated with higher rates of early recurrence, while ECMO use and timing of repair with ECMO were not. Type of study Treatment study. Level of evidence II.

Contains fulltext : 178192.pdf (Publisher’s version ) (Closed access) BACKGROUND: Congenital diaphragmatic hernia (CDH) is a lethal birth defect, which occurs in 1:2000-3000 live births. Bilateral CDH is a rare form (1%), with a high mortality. This study presents the outcomes of the largest cohort of bilateral CDH patients. METHODS: The records of patients with bilateral CDH from the Congenital Diaphragmatic Hernia Registry born between 1995 and 2015 were retrospectively analyzed to identify parameters associated with mortality. RESULTS: Eighty patients with a bilateral CDH were identified. Overall mortality was 74% (n=59). Apgar scores at 1 and 5min were statistically lower in the non-survivors compared to the survivors (median 3.0 and 5.0, versus 6.5 and 8.0, respectively, p

Epoprostenol (also called prostaglandin, PGI) is used for pulmonary hypertension in newborns with congenital diaphragmatic hernia (CDH) in some centers. The effects of PGI on survival in newborns with CDH were examined.A retrospective analysis of the Congenital Diaphragmatic Hernia Study Group registry between 2007and 2011 was performed. Patient-level logistic regression was applied in a subset of 29 hospitals with a history of PGI use to relate the probability of death to the use of PGI within 7 days of surgery after controlling for ethnicity, prenatal diagnosis, prenatal steroids, CDH defect, chromosomal abnormalities, liver location, complex cardiac anomalies, 5-minute Apgar score, and operative day of life. This analysis was repeated after excluding 50% of the patients with the lowest probabilities (0.042 mean propensity score) of receiving treatment. To reduce confounding by indication, a separate mixed effects logistic regression analysis was performed in 58 hospitals to relate the hospital-level mortality to the proportion of patients administered PGI after controlling for hospital-level covariates.Epoprostenol was administered within 7 days of surgery for 80 (7.3%) of these subjects. Epoprostenol use was associated with higher mortality (odds ratio [OR] 4.39, 95% confidence interval [CI] 2.04-9.48) in the patient-level analyses without covariate adjustment. The direct association of epoprostenol use with mortality was partially reduced after covariate adjustment (adjusted OR 2.24, 95% CI 0.95-5.29, p = 0.07), and further attenuated after both covariate adjustment and restriction of the analysis to patients with propensity scores 0.042 (adjusted OR 1.71, 95% CI 0.68-4.29, p = 0.26). A total of 182 of the 1,639 patients included in the center-level dataset died after 7 days of operation. There was no statistically significant association of mortality with the proportion of patients administered epoprostenol in hospital-level analysis (adjusted OR 0.63, 95% CI 0.34-1.17 per 25% increase, p = 0.15).The discrepancy of results between the hospital and patient-level analyses suggests that the association of mortality and PGI in the patient-level analyses resulted from bias by indication. Hospital-level results provided no evidence of a benefit of PGI use on survival, but may have failed to detect a true benefit due to limited statistical power. Further use of PGI in this population should only be recommended after rigorous evaluation, such as a randomized controlled trial.

The minimally invasive surgery (MIS) approach for congenital diaphragmatic hernia (CDH) repair remains controversial. Our objective was to compare outcomes and complications of the MIS and open approaches, with risk-stratification of patients based on defect size and key patient characteristics.The multinational CDH Study Group (CDHSG) registry was queried for the period from 2007 to 2015. Patient demographics and operative details, including the CDHSG Staging System defect size (A to D), were reviewed. Open cases consisted of laparotomy and thoracotomy; MIS repairs included laparoscopy and thoracoscopy. Outcomes included length of stay (LOS) for patients surviving to discharge, hernia recurrence, and adhesive small bowel obstruction (SBO) requiring surgery. Regression analyses were performed. Odds ratios (ORs) with 95% CIs were derived.A total of 3,067 CDH patients underwent open (n = 2,579; 84%) or MIS (n = 488; 16%) repair. Patients undergoing open repair were more likely to be diagnosed prenatally, be premature, have lower 5-minute Apgar scores, and have major cardiac anomalies (all p0.001). Among MIS repairs, 79% were low risk (size A and B) defects vs 50% among open repairs (p 0.001). Patients undergoing MIS repair experienced shorter overall median LOS, higher recurrence rates, and fewer SBO. With multivariable regression adjusting for defect size and key patient characteristics, an MIS approach was significantly associated with decreased LOS (mean -13.4 days; 95% CI -18 to -8.8 days), increased recurrences (OR 3.10; 95% CI 1.91 to 5.04), and decreased SBO (OR 0.19; 95% CI 0.06 to 0.60).After risk-stratification of CDH patients, an MIS approach was independently associated with decreased LOS and SBO, but higher recurrence rates.

PURPOSE The aim of our study was to elucidate if the defect size reflects the magnitude of the developmental defect in patients with CDH. METHODS All patients recorded in the CDH Study Group registry between January 1, 2007, and December 31, 2010, and with defect classification were included in the study. They were divided according to defect size (A-D) and compared for: gestational age (GA), birth weight (BW), number of abnormal organ systems, prevalence of associated anomalies, cardiovascular malformations (CVM), chromosomal anomalies, liver in the chest, and hernia sac. RESULTS A total of 1350 of 1778 patients had defect classification: 173 A, 557 B, 438 C, and 182 D. Mortality rate was 0.6%, 5.3%, 22.6%, and 45.6% in group A, B, C, and D, respectively, (p

Purpose Chylothorax is a known complication in neonates after congenital diaphragmatic hernia (CDH) repair. This report uses a large international registry to evaluate risk factors, treatment, morbidity, and survival associated with chylothorax in a prospective cohort of neonates after CDH repair. Methods From January 2007 to January 2010, live-born neonates with repaired, unilateral CDHs were evaluated from a prospective database for chylothorax development. Chylothorax was diagnosed based on pleural fluid examination. Study variables included patient characteristics, CDH defect and disease severity characteristics, chylothorax treatment, and survival. In addition, the temporal relationship between timing of CDH repair and extracorporeal membrane oxygenation (ECMO) therapy was evaluated as a risk factor for chylothorax. Univariate and multivariate regression analyses were utilized. Results Among the 1383 patients evaluated, chylothorax was diagnosed in 4.6% of the cohort. Patch repair and ECMO were statistically significant risk factors for chylothorax. The odds of developing a chylothorax were significantly increased in patients with CDH repair on ECMO (aOR 2.6; 95% CI: 1.3–4.9) or after ECMO (aOR 3.1; 95% CI: 1.7–5.8). Most chylothoraces (83.1%) were successfully treated without surgery. Chylothorax patients had significant morbidity including increased oxygen use at 30days and longer length of stay. Survival was not significantly affected by chylothorax. Conclusions Chylothorax is a known but uncommon complication of neonatal CDH repair. In this very large series of chylothorax in association with CDH, major risk factors appear to be related to increased disease severity with the highest risk in patients repaired on or after ECMO. Chylothoraces usually improve with conservative therapy and lead to significant morbidity but not increased mortality.

Importance Inhaled nitric oxide (iNO) is an expensive, commonly used therapy among patients with congenital diaphragmatic hernia (CDH); however, data to support its ongoing use in this patient population are lacking. Objective To describe the spectrum of iNO use among patients with CDH and its association with pulmonary hypertension (pHTN) and mortality. Design, Setting, and Participants A review was conducted of prospectively collected patient data in the Congenital Diaphragmatic Hernia Study Group registry between January 1, 2007, and December 31, 2014, from 70 participating centers in 13 countries. A total of 3367 newborn infants diagnosed with CDH and entered into the registry were reviewed. On the basis of echocardiogram data, pHTN was defined as right ventricular systolic pressure greater than or equal to two-thirds of the systemic systolic pressure. Propensity score and regression analyses were performed. Intervention Use of iNO. Main Outcomes and Measures Variability in iNO use and its association with pHTN and mortality. These outcomes were formulated prior to data evaluation. Results Sixty-eight (97.1%) centers used iNO. Of 3367 patients with CDH (1366 [40.6%] females; median estimated gestational age, 38 weeks; range, 23-42 weeks), a total of 2047 (60.8%) received iNO; the mean percentage of those receiving iNO per center was 62.3% (range, 0%-100%). Median iNO dose and duration were 20 (range, 0.1-80) ppm and 8 (range, 0-100) days. Of the 2174 infants with pHTN, 1613 infants (74.2%) received iNO. Of the 943 infants without pHTN, 343 infants (36.4%) were treated with iNO. Based on propensity score analysis incorporating 10 clinically relevant variables, iNO use was significantly associated with increased mortality (average treatment effect on the treated: 0.15; 95% CI, 0.10-0.20). Conclusions and Relevance Inhaled nitric oxide use is common but highly variable among centers, and 36% of patients without pHTN received iNO therapy. Based on data from 70 centers, iNO use in patients with CDH may be associated with increased mortality. Future efforts should be directed toward data-driven standardization of iNO use to ensure cost-effective practices.

OBJECTIVE To evaluate if the delivery mode of infants with prenatally diagnosed congenital diaphragmatic hernia (CDH) affects the outcome. STUDY DESIGN Data from the CDH Study Group database of infants with prenatal diagnosis between 2001-2015 were divided into four delivery mode groups: vaginal spontaneous, vaginal induced, elective caesarean section, and emergent caesarean section. Outcomes were analyzed in relation to the time of day of delivery and the gestational age at birth. RESULTS A total of 3906 cases of prenatally diagnosed CDH were assessed, with an overall survival of 64%. There were no differences in patient characteristics, requirement for extracorporeal membrane oxygenation, length of hospital stay or intubation, requirement for O2 at 30 days or overall survival. The time of day at birth did not affect the outcome. There was no difference in outcome between the different delivery modes at similar gestational age (GA) at birth, with worse outcomes at lower GA. CONCLUSIONS Neither the mode nor time of delivery seems to affect the overall outcome for patients with prenatally diagnosed CDH. Outcome is strongly associated with the GA at birth.

To analyze operative repair, extracorporeal membrane oxygenation (ECMO) and survival rates based on highest pre-ductal oxygen saturation (Pre-O(2)SAT) in a large infant cohort reported to Congenital Diaphragmatic Hernia Study Group Registry between 2000 and 2010.Analyzed data included gestational age, birth weight, defect side and size, repair, ECMO use, survival and highest reported PaO(2) and Pre-O(2)SAT in first 24 h of life. We excluded 614 infants due to severe anomaly. Pre-O(2)SAT data were available for 1672 infants.Among infants with highest Pre-O(2)SAT value85%, survival (24/105=23%) and repair (55/105=52%) rates were significantly decreased compared with infants with higher values. Survival increased to 44% for infants with highest Pre-O(2)SAT85% who underwent operative repair. Of these, 83% (20/24) required ECMO support compared with 15% (144/961) of survivors with Pre-O(2)SAT99% (P0.001). The lowest reported Pre-O(2)SAT with survival was 32% and for survival without ECMO was 52%.A reported highest Pre-O(2)SAT85% in the first 24 h of life was not uniformly fatal; but survival of infants with Pre-O(2)SAT85% was associated with high ECMO use and prolonged hospitalization.

Congenital diaphragmatic hernia (CDH) is a rare anomaly with an incidence between 1/2,500 and 1/3,000 live births. The rarity of the disease makes it difficult to design powerful studies leading to accurate and meaningful evidence. For rare diseases, the development of multicenter international registries may help in collecting data and give an overall picture of the disease. In this review, we will describe the development of the CDH study group, we will describe its work methodology, and the results obtained since its birth in 1995.

Congenital diaphragmatic hernia (CDH) remains a significant cause of death in newborns. With advances in neonatal critical care and ventilation strategies, survival in the term infant now exceeds 80% in some centers. Although prematurity is a significant risk factor for morbidity and mortality in most neonatal diseases, its associated risk with infants with CDH has been described poorly. We sought to determine the impact of prematurity on survival using data from the Congenital Diaphragmatic Hernia Registry (CDHR).Prospectively collected data from live-born infants with CDH were analyzed from the CDHR from January 1995 to July 2009. Preterm infants were defined as37 weeks estimated gestational age at birth. Univariate and multivariate logistic regression analysis were performed.During the study period, 5,069 infants with CDH were entered in the registry. Of the 5,022 infants with gestational age data, there were 3,895 term infants (77.6%) and 1,127 preterm infants (22.4%). Overall survival was 68.7%. A higher percentage of term infants were treated with extracorporeal membrane oxygenation (ECMO) (33% term vs 25.6% preterm). Preterm infants had a greater percentage of chromosomal abnormalities (4% term vs 8.1% preterm) and major cardiac anomalies (6.1% term vs 11.8% preterm). Also, a significantly higher percentage of term infants had repair of the hernia (86.3% term vs 69.4% preterm). Survival for infants that underwent repair was high in both groups (84.6% term vs 77.2% preterm). Survival decreased with decreasing gestational age (73.1% term vs 53.5% preterm). The odds ratio (OR) for death among preterm infants adjusted for patch repair, ECMO, chromosomal abnormalities, and major cardiac anomalies was OR 1.68 (95% confidence interval [CI], 1.34-2.11).Although outcomes for preterm infants are clearly worse than in the term infant, more than 50% of preterm infants still survived. Preterm infants with CDH remain a high-risk group. Although ECMO may be of limited value in the extremely premature infant with CDH, most preterm infants that live to undergo repair will survive. Prematurity should not be an independent factor in the treatment strategies of infants with CDH.

BACKGROUND: Severe congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO) is associated with high mortality. Timing of CDH repair relative to ECMO therapy remains controversial. Our hypothesis was that survival would significantly differ between those who underwent repair during ECMO and those who underwent repair after ECMO therapy. METHODS: We examined de-identified data from the CDH Study Group (CDHSG) registry from 1995–2005 on patients who underwent repair and ECMO therapy (n=636). We used Cox regression analysis to assess differences in survival between those repaired during and after ECMO. RESULTS: Five covariates were significantly associated with mortality: timing of repair relative to ECMO (P=0.03), defect side (P=0.01), ECMO run length (P

Background Late-presenting congenital diaphragmatic hernia (CDH) is a rare subset of CDH, most of the information derived from small series or case reports. The aim of this study was to document the clinical manifestations of late-presenting CDH using a large multicenter database. Methods Information about late-presenting CDH (diagnosed at later than 30 days of age) was identified from the database of the CDH Study Group (3098 cases collected during 1995-2004) and reviewed retrospectively. Results Seventy-nine cases (2.6%) from 30 centers met the inclusion criteria. Seven cases had a Morgagni hernia. There were 50 males (65%) and 27 females (35%). The mean age at diagnosis was 372 days (32 days to 15 years). Major associated anomalies (10 cardiac and 7 chromosomal abnormalities) were identified in 12 cases (15%). Presenting symptoms were respiratory in 20 (43%), gastrointestinal in 15 (33%), both in 6 (13%), and none (asymptomatic) in 5 (11%). The hernia was left-sided in 53 (69%), right-sided in 21 (27%), and central or bilateral in 3 (4%). Patients with gastrointestinal symptoms invariably had left-sided hernias (n = 19), whereas patients with respiratory symptoms (n = 24) seemed equally likely to have right- or left-sided lesions. A primary repair without patch was done in all cases with 100% survival. Conclusions Presenting symptoms of late-onset CDH can be respiratory or gastrointestinal, but presentation with gastrointestinal problems was more common in left-sided hernias, whereas respiratory symptoms predominated in right-sided lesions. The prognosis is excellent once the correct diagnosis is made.

To evaluate the impact of treated extra-pulmonary metastatic disease on overall (OS) and event-free survival (EFS) for pediatric osteosarcoma patients undergoing pulmonary metastatectomy.We retrospectively reviewed pediatric patients who were treated for osteosarcoma at our institution from 2001 to 2011 and received pulmonary metastatectomy (n=76). We compared OS and EFS between patients with metastases limited to the lungs (Group A, n=58) to those with treated extra-pulmonary metastases (Group B, n=18) at the time of first pulmonary metastatectomy.The estimated median OS and EFS from first pulmonary metastatectomy were 2.0years (95% CI 1.5-2.8years) and 5.5months (95% CI 3.0-8.1months), respectively. Median OS was significantly greater for Group A (2.6years, 95% CI 1.9-3.8) compared to Group B (0.9years, 95% CI 0.6-1.5) (log rank p=0.0001). Median EFS was significantly greater for Group A (7.9months, 95% CI 5.0-10.7) compared to Group B (1.6months, 95% CI 0.8-2.7) (log rank p0.0001). Independent predictors of OS included extra-pulmonary metastatic disease at the time of first thoracotomy, bilateral pulmonary metastases, and4 nodules resected at first thoracotomy (all p0.001).Osteosarcoma patients with treated extra-pulmonary metastatic disease at the time of pulmonary metastatectomy have significantly worse survival compared to those with disease limited to the lungs.

Background Congenital diaphragmatic hernia (CDH) remains a significant cause of death in newborns and, despite improved outcomes with multimodality therapies, optimal timing of repair remains undefined. We sought to evaluate the influence of surgical timing on patient outcomes and hypothesized that delayed repair does not improve survival in CDH. Methods Prospectively collected data from 1,385 CDH Registry infants without preoperative extracorporeal membrane oxygen therapy (ECMO) were evaluated. Patients were stratified by timing of repair: Day of life (DOL) 0–3 (group 1), 4–7 (group 2), or >8 (group 3), and the effect of surgical timing on mortality was determined by logistic regression and risk-adjusted for severity of illness. Results The unadjusted odds ratio (OR) for mortality increased significantly with delayed repair (group 2, 1.73 [95% CI, 1.00–2.98; group 3, 3.42 [95% CI, 1.97–5.96]). However, when adjusted for severity of illness, delay in repair did not predict increased mortality (group 2, 1.2 [95% CI, 0.7–2.2]; group 3, 1.4 [95% CI, 0.8–2.6]), nor did it portend an increased need for postoperative ECMO (group 2, 1.1 [95% CI, 0.5–2.4]; group 3, 0.5 [95% CI, 0.2–1.4]). Conclusion After adjustment for known risk factors, the timing of CDH repair in low-risk infants does not seem to influence mortality. However, specific clinical parameters guiding timing of elective CDH repair remain unknown.

Background/purpose Congenital diaphragmatic hernia (CDH) remains a significant cause of neonatal death. A wide spectrum of disease severity and treatment strategies makes comparisons challenging. The objective of this study was to create a standardized reporting system for CDH. Methods Data were prospectively collected on all live born infants with CDH from 51 centers in 9 countries. Patients who underwent surgical correction had the diaphragmatic defect size graded (A–D) using a standardized system. Other data known to affect outcome were combined to create a usable staging system. The primary outcome was death or hospital discharge. Results A total of 1,975 infants were evaluated. A total of 326 infants were not repaired, and all died. Of the remaining 1,649, the defect was scored in 1,638 patients. A small defect (A) had a high survival, while a large defect was much worse. Cardiac defects significantly worsened outcome. We grouped patients into 6 categories based on defect size with an isolated A defect as stage I. A major cardiac anomaly (+) placed the patient in the next higher stage. Applying this, patient survival is 99% for stage I, 96% stage II, 78% stage III, 58% stage IV, 39% stage V, and 0% for non-repair. Conclusions The size of the diaphragmatic defect and a severe cardiac anomaly are strongly associated with outcome. Standardizing reporting is imperative in determining optimal outcomes and effective therapies for CDH and could serve as a benchmark for prospective trials.

BACKGROUND AND OBJECTIVE: Survival for infants with congenital diaphragmatic hernia (CDH) has gradually improved, yet substantial burden of disease remains. Although larger CDH defect sizes increase mortality, the association between defect size and morbidity has not been reported. Our objective was to evaluate the association of defect size with pulmonary, neurologic, and gastrointestinal morbidity at the time of hospital discharge. METHODS: An international, prospective cohort study was performed. Patient demographics, intraoperative defect size, and clinical outcomes were reviewed. The primary outcome was morbidity at the time of discharge, which entailed supplemental oxygen requirement, abnormal neurologic clinical and radiographic findings, gastroesophageal reflux, supplemental nutrition, or pulmonary-, neurologic-, or gastrointestinal-related medications. RESULTS: A total of 3665 patients were included in the study cohort. Overall survival was 70.9%, and 84.0% of survivors were discharged from the hospital (16.0% transferred). Median age at discharge was 38 days (interquartile range [IQR] 23–69) and ranged from 22 (IQR 16–32) days for “A” (smallest) defects to 89 (IQR 64–132) days for “D” (largest) defects ( P n = 660, 30.2%), neurologic ( n = 446, 20.4%), or gastrointestinal ( n = 1348, 61.7%) morbidities, and multiple morbidities were diagnosed in 701 (34.7%) patients. On multivariable regression analyses incorporating key patient characteristics, defect size was consistently the greatest predictor of overall morbidity, hospital length of stay, and duration of ventilation. CONCLUSIONS: Infants with CDH are commonly discharged with ≥1 major morbidities. The size of the diaphragmatic defect appears to be the most reliable indicator of a patient’s hospital course and discharge burden of disease.

To evaluate the impact of associated heart defects on outcomes to discharge, and identify factors affecting survival of all infants born with congenital diaphragmatic hernia (CDH) in last decade using Congenital Diaphragmatic Hernia Study Group data.This was a retrospective review of all infants with CDH enrolled in Congenital Diaphragmatic Hernia Study Group database from January 2000 to December 2010. The study cohort was divided into 3 groups (GRP): GRP 1, CDH with major heart defects; GRP 2, CDH with minor heart defects; and GRP 3, CDH with no reported heart defects.The 4268 enrolled infants included 345 (8%) in GRP 1, 412 (10%) in GRP 2, and 3511 (82%) in GRP 3. Survival was significantly lower in GRP 1 compared with GRP 2 and GRP 3 (36% vs 73%). In GRP 1, the most common defects were left heart obstructive lesions (34%). Survival was lowest in infants with transposition of great arteries (0%) and single ventricle physiology (16%). There was no change in survival rate for any group between 2000-2005 and 2006-2010. In GRP 1, factors that predicted lower survival were birth weight2.5 kg, associated noncardiac anomalies, single ventricle physiology, no sildenafil therapy, no CDH repair, and no cardiac repair.Survival is significantly lower in patients with CDH and major heart defects compared with patients with minor or no heart defects. Outcomes of newborns with CDH and major heart defects have not improved over the last decade.

Background: Congenital diaphragmatic hernia (CDH) is associated with a mortality rate of 10–35% in live-born infants. Moreover, CDH survivors have a substantial risk of developing long-term pulmonary sequelae, such as bronchopulmonary dysplasia (BPD). Objectives: This study aims to evaluate risk factors associated with BPD and mortality in neonates with CDH, with particular focus on the initial ventilation mode. Methods: Eligible for inclusion were live-born infants with CDH born from 2001 through 2006 at the centers participating in the CDH Study Group. BPD (defined as oxygen dependency at day 30) and/or mortality by day 30 served as the primary endpoint. Results: A total of 2,078 neonates were included in the analysis. At day 30, 56% of the patients had either died or met the criteria for BPD. In infants who survived until day 30, the prevalence of BPD was 41%. The overall mortality rate was 31%. High-frequency oscillatory ventilation as initial ventilation mode, a right-sided defect, a prenatal diagnosis, a lower Apgar score at 5 min, a cardiac anomaly, a chromosomal anomaly and a lower gestational age were all associated with BPD and/or mortality by day 30. Conclusions: Despite improvements in neonatal care, the rates of BPD and early mortality in newborns with CDH are still considerable. Several important risk factors for a worse outcome are reported in this nonrandomized prospective observational study.

OBJECTIVES. The goal of the study was to test the hypothesis that infants with known congenital diaphragmatic hernias born at early term gestation (37–38 weeks) rather than later (39–41 weeks) had greater survival rates and less extracorporeal membrane oxygenation use. Primary outcomes were survival to hospital discharge or transfer and extracorporeal membrane oxygenation use. METHODS. A retrospective cohort study of term infants with prenatal diagnoses of congenital diaphragmatic hernia was performed with the Congenital Diaphragmatic Hernia Study Group Registry of patients with congenital diaphragmatic hernias who were treated between January 1995 and December 2006. RESULTS. Among 628 term infants at 37 to 41 weeks of gestation who had prenatal diagnoses of congenital diaphragmatic hernia and were free of major associated anomalies, early term birth (37 vs 39–41 weeks) and greater birth weight were associated independently with survival, whereas black race was related inversely to survival. Infants born at early term with birth weights at or above the group mean (3.1 kg) had the greatest survival rate (80%). Among infants born through elective cesarean delivery, infants born at 37 to 38 weeks of gestation, compared with 39 to 41 weeks, had less use of extracorporeal membrane oxygenation (22.0% vs 35.5%) and a trend toward a greater survival rate (75.0% vs 65.8%). CONCLUSIONS. The timing of delivery is an independent, potentially important factor in the consideration of elective delivery for infants diagnosed prenatally as having congenital diaphragmatic hernias. Among fetuses with prenatally diagnosed congenital diaphragmatic hernias and without major associated anomalies, early term delivery may confer advantage.