Severity Of Congenital Diaphragmatic Hernia Research Articles

Bleeding Complications between Bivalirudin and Heparin for ECMO in Neonates with CDH.

Infants with congenital diaphragmatic hernia (CDH) who undergo repair while on extracorporeal membrane oxygenation (ECMO) are at risk for developing post-operative bleeding complications. Balanced anticoagulation is critical to maintain ECMO flow and avoid bleeding. Heparin has historically been our first-line anticoagulant; however, recently, we transitioned to bivalirudin, a direct thrombin inhibitor. The objective of this pilot study was to compare post-operative surgical bleeding complications between the two groups. A single center retrospective cohort study of patients who underwent CDH repair while on ECMO between 2008-2023 was performed. Neonates were stratified based on type of anticoagulant initiated after CDH repair. Outcomes included bleeding requiring surgical re-operation, intracranial hemorrhage, volume of blood products transfused, number of circuit changes, days on ECMO, and overall survival. Among 62 infants with CDH who underwent repair on ECMO, 44 (71%) were managed post-CDH repair with heparin and 18 (29%) with bivalirudin. Demographics and prenatal predictors of CDH severity were not significantly different between the two cohorts. One (5.6%) neonate managed with bivalirudin required re-operation following CDH repair for a bleeding complication compared to 17 (38.6%) managed with heparin (p=0.022). In addition, the bivalirudin cohort required half of the total blood product volume compared to the heparin cohort (p=0.020). Despite these benefits, there were no significant differences between groups for incidence of intracranial hemorrhage, number of circuit changes, days on ECMO, and overall survival. Anticoagulation with bivalirudin in neonates who underwent CDH repair while on ECMO was associated with decreased surgical bleeding complications and less total blood product transfused. This pilot analysis is the first to directly compare heparin to bivalirudin and stresses the importance of a randomized trial.

Association between Time with Open Ductus Arteriosus and Outcomes in Congenital Diaphragmatic Hernia

Introduction: While a patent ductus arteriosus (PDA) helps offload the right ventricle in the acute congenital diaphragmatic hernia (CDH)-associated pulmonary hypertension, its role on long-term outcomes in CDH has not been investigated. Our objective was to examine associations of the PDA with long-term clinical outcomes in CDH. Methods: A single-center retrospective descriptive study of 122 CDH patients dichotomized by duration with PDA, as ≤14 versus >14 postnatal days (PND) and ≤30 versus >30 PND. Fisher’s exact test, Wilcoxon rank-sum test, and multiple linear and logistic regression analyses were used for analyses. Results: In unadjusted and adjusted for CDH severity comparisons, patients with PDA >14 PND and >30 PND had a higher risk of death, longer length of stay, mechanical ventilation duration, and need for tracheostomy, diuretics, and PH medications at discharge. Conclusion: A PDA beyond the newborn period is associated with adverse outcomes in infants with CDH.

Prevalence of Gastroesophageal Reflux Disease in Congenital Diaphragmatic Hernia Survivors From Infancy to Adulthood

BackgroundGastroesophageal reflux disease (GERD) is a common comorbidity associated with congenital diaphragmatic hernia (CDH), with reported cases of Barrett's esophagus (BE) and esophageal adenocarcinoma before the age of 25. The prevalence and natural course of GERD in CDH survivors remain uncertain due to variations in diagnostic methods. We aimed to analyse the GERD prevalence from infancy through young adulthood. MethodsWe retrospectively analyzed pH-impedance measurements and endoscopic findings in 96 CDH survivors evaluated as routine care using well established clinical protocols.GERD was defined as an abnormal acid exposure time for pH-MII measurements and as presence of reflux esophagitis or BE at upper endoscopy. Clinical data including symptoms at time of follow-up and use of antireflux medication were collected. ResultsGERD prevalence remained consistently low (≤10%) across all age groups, yet many patients experienced GER symptoms. Histological abnormalities were observed in 80% of adolescents and young adults, including microscopic esophagitis in 50%. BE was diagnosed in 7% before the age of 18, all had GER symptoms. CDH severity, anatomy at the time of CDH correction, alcohol usage, and smoking did not emerge as significant risk factors for GERD. ConclusionsGiven the low GERD prevalence in CDH survivors, a symptom-driven approach to diagnosis and follow-up is warranted. We advise long-term follow-up for all adult patients due to the early onset of BE and the limited evidence available. The longitudinal course and impact of GERD on other long-term CDH-related comorbidities should be explored in larger cohorts. Level of EvidenceNot applicable

Disease severity impacts perceived quality of life in congenital diaphragmatic hernia: a prospective observational study

BackgroundWhile research indicates comparable quality of life (QOL) in congenital diaphragmatic hernia (CDH) and healthy populations, the effect of CDH severity on patients’ health perceptions remains unexplored. We aimed to...

Congenital Diaphragmatic Hernia Patients with Left Heart Hypoplasia and Left Ventricular Dysfunction Have Highest Odds of Mortality

To describe the scope of left ventricular (LV) dysfunction and left heart hypoplasia (LHH) in infants with congenital diaphragmatic hernia (CDH), to determine associations with CDH severity, and to evaluate the odds of extracorporeal membrane oxygenation (ECMO) and death with categories of left heart disease. Demographic and clinical variables were collected from a single-center, retrospective cohort of patients with CDH from January 2017 through May 2022. Quantitative measures of LV function and LHH were prospectively performed on initial echocardiograms. LHH was defined as ≥2 of the following: z score≤-2 of any left heart structure or LV end-diastolic volume <3mL. LV dysfunction was defined as shortening fraction <28%, ejection fraction <60%, or global longitudinal strain <20%. The exposure was operationalized as a 4-group categorical variable (LV dysfunction +/-, LHH +/-). Logistic regression models evaluated associations with ECMO and death, adjusting for CDH severity. One hundred eight-two patients (80.8% left CDH, 63.2% liver herniation, 23.6% ECMO, 12.1% mortality) were included. Twenty percent demonstrated normal LV function and no LHH (LV dysfunction-/LHH-), 37% normal LV function with LHH (LV dysfunction-/LHH+), 14% LV dysfunction without LHH (LV dysfunction+/LHH-), and 28% both LV dysfunction and LHH (LV dysfunction+/LHH+). There was a dose-response effect between increasing severity of left heart disease, ECMO use, and mortality. LV dysfunction+/LHH+infants had the highest odds of ECMO use and death, after adjustment for CDH severity [OR (95% CI); 1.76 (1.20, 2.62) for ECMO, 2.76 (1.63, 5.17) for death]. In our large single-center cohort, patients with CDH with LV dysfunction+/LHH+had the highest risk of ECMO use and death.

Congenital diaphragmatic hernia: automatic lung and liver MRI segmentation with nnU-Net, reproducibility of pyradiomics features, and a machine learning application for the classification of liver herniation

Prenatal assessment of lung size and liver position is essential to stratify congenital diaphragmatic hernia (CDH) fetuses in risk categories, guiding counseling, and patient management. Manual segmentation on fetal MRI provides a quantitative estimation of total lung volume and liver herniation. However, it is time-consuming and operator-dependent. In this study, we utilized a publicly available deep learning (DL) segmentation system (nnU-Net) to automatically contour CDH-affected fetal lungs and liver on MRI sections. Concordance between automatic and manual segmentation was assessed by calculating the Jaccard coefficient. Pyradiomics standard features were then extracted from both manually and automatically segmented regions. The reproducibility of features between the two groups was evaluated through the Wilcoxon rank-sum test and intraclass correlation coefficients (ICCs). We finally tested the reliability of the automatic-segmentation approach by building a ML classifier system for the prediction of liver herniation based on support vector machines (SVM) and trained on shape features computed both in the manual and nnU-Net-segmented organs. We compared the area under the classifier receiver operating characteristic curve (AUC) in the two cases. Pyradiomics features calculated in the manual ROIs were partly reproducible by the same features calculated in nnU-Net segmented ROIs and, when used in the ML procedure, to predict liver herniation (both AUC around 0.85). Conclusion: Our results suggest that automatic MRI segmentation is feasible, with good reproducibility of pyradiomics features, and that a ML system for liver herniation prediction offers good reliability. Trial registration: https://clinicaltrials.gov/ct2/show/NCT04609163?term=NCT04609163&draw=2&rank=1; Clinical Trial Identification no. NCT04609163.What is Known:• Magnetic resonance imaging (MRI) is crucial for prenatal congenital diaphragmatic hernia (CDH) assessment. It enables the quantification of the total lung volume and the extent of liver herniation, which are essential for stratifying the severity of CDH, guiding counseling, and patient management.• The manual segmentation of MRI scans is a time-consuming process that is heavily reliant upon the skill set of the operator.What is New:• MRI lung and liver automatic segmentation using the deep learning nnU-Net system is feasible, with good Jaccard coefficient values and satisfactory reproducibility of pyradiomics features compared to manual results.• A feasible ML system for predicting liver herniation could improve prenatal assessments and CDH patient management.

Prenatal Predictors of Survival, Pulmonary Hypertension, and ECMO in Isolated CDH Undergoing Expectant Management, A Systematic Review and Meta-analysis

Background and Hypothesis: Congenital diaphragmatic hernia (CDH) is a severe developmental defect affecting 1-4 per 10,000 births, characterized by left/right-sided defect, or mixed with herniation of abdominal contents into thorax, with resultant lung hypoplasia and persistent pulmonary hypertension (PHTN). The study investigates prenatal predictors of survival to hospital discharge, PHTN and the need for ECMO in fetuses with isolated CDH undergoing prenatal expectant management.&#x0D; Project Methods: We performed a systematic literature review on prenatal diagnostic tests in fetuses with isolated CDH undergoing expectant management. Primary outcomes included survival-to-hospital discharge, persistent PHTN within 28 days, and ECMO need. Newcastle Ottawa Scale assessed quality of studies. Meta-analysis performed when two or more studies reported on the same test. Subgroup analysis performed according to CDH side.&#x0D; Results: 161 full-text articles between 2000-2022 were assed for eligibility; 48 met inclusion criteria. 45 reported on survival, 12 on ECMO need, 8 on PHTN; quality of studies was moderate. Studies included were retrospective (81%) or prospective (19%) regarding fetuses undergoing expectant management (77%), or mixed tracheal occlusion and expectant management (20%). Most studies included mixed (41%) or left-sided (47%) CDH. Survival predicted by TFLV, o/e-TFLV, o/e-TFLV &lt;30%, LiTR, o/e-LHR, o/e-LHR &lt;25%, percentage herniated liver, MSA, stomach position in mid-chest, and liver up. ECMO need predicted by o/e-TFLV, o/e-LHR, and PPLV. These results were confirmed through subgroup analysis of only left-sided lesions. PHTN was predicted by presence of intrathoracic liver (OR-1.96, 95%CI 1.14,3.37, I2-0%); this was not significant after left-sided subgroup analysis.&#x0D; Conclusion and Potential Impact: In fetuses with CDH, FLV and presence/percentage of intrathoracic liver predict survival. FLV measurements predict ECMO need. Presence of intrathoracic liver may predict persistent PHTN; further studies are needed. Accurate prognostication of CDH severity would aid patient triage, resource mobilization, and identification of high-risk CDH infants in need of advanced treatment including ECMO or identification of candidates for fetal intervention procedures.

Muscle Flap Technique Safe for On-ECMO Congenital Diaphragmatic Hernia Repair

IntroductionProsthetic patches (patch) and muscle flaps (flap) are techniques used for repair of congenital diaphragmatic hernia (CDH) with a large defect unamenable to primary closure. We hypothesized that the flap technique for CDH repair while on extra-corporeal membrane oxygenation (on-ECMO) would have decreased bleeding complications compared to patch due to the hemostatic advantage of native tissue. MethodsA single-center retrospective comparative study of patients who underwent on-ECMO CDH repair between 2008 and 2022 was performed. ResultsFifty-two patients met inclusion criteria: 18 patch (34.6%) and 34 flap (65.4%). There was no difference in CDH severity between groups. On univariate analysis, reoperation for surgical bleeding was lower following flap repair compared to patch (23.5% vs 55.6%, respectively; p = 0.045), 48-h postoperative blood product transfusion was lower after flap repair (132 mL/kg vs 273.5 mL/kg patch; p = 0.006), and two-year survival was increased in the flap repair group compared to patch (53.1% vs 17.7%, respectively; p = 0.036). On multivariate analysis adjusting for CDH side, day on ECMO repaired, and day of life CDH repaired, flap repair was significantly associated with lower five-day postoperative packed red blood cell transfusion amount, improved survival to hospital discharge, and improved two-year survival. ConclusionsOur experience suggests that the muscle flap technique for on-ECMO CDH repair is associated with reduced bleeding complications compared to prosthetic patch repair, which may in part be responsible for the improved survival seen in the flap repair group. These results support the flap repair technique as a favored method for on-ECMO CDH repair. Level of EvidenceLevel III.

Prenatal Brain Maturation is Delayed in Neonates with Congenital Diaphragmatic Hernia

To assess brain development in fetuses with congenital diaphragmatic hernia (CDH) using a fetal Total Maturation Score (fTMS). This is a retrospective cohort study using data from a single-center, clinical registry. Neonates with an antenatal diagnosis of CDH between 2014 - 2020 and prenatal brain magnetic resonance imaging (n = 48) were included. We compared our study sample with historical healthy controls (n = 48). The relationship between fTMS and gestational age, as well as the association between fTMS and key prenatal variables and placental pathologic findings, were evaluated. Compared with healthy controls, neonates with CDH had a significant delay in fTMS (p-value <0.001). Within the CDH cohort, there was no significant difference in fTMS based on CDH severity, intrathoracic liver position, right versus left CDH, sex, presence of abnormal echocardiogram findings, treatment with extracorporeal membrane oxygenation, or in-hospital mortality. Placentas of neonates with CDH had a high proportion of fetal vascular malperfusion (56%) and chronic inflammation (67%), and relatively large placentas had a protective effect on prenatal brain maturation (p-value = 0.025). Prenatal brain maturation in neonates with CDH is delayed. Placental pathology may influence fetal brain development. The etiology and clinical impact of prenatal brain immaturity in neonates with CDH warrant further investigation.

First-year growth trajectory and early nutritional requirements for optimal growth in infants with congenital diaphragmatic hernia: a retrospective cohort study

ObjectiveTo describe the growth trajectory of children with congenital diaphragmatic hernia (CDH) during the first year, to assess the risk factors for growth failure (GF) at 1 year and to...

The Association between the First Cry and Clinical Outcomes in CDH Neonates: A Retrospective Study.

Congenital diaphragmatic hernia (CDH) is a life-threatening condition characterized by the herniation of abdominal organs into the thorax, resulting in hypoplastic lungs and pulmonary hypertension. The impact of the first cry, a crucial event for lung transition during birth, on CDH patients remains unclear. This study investigated the impact of the first cry during birth on CDH patient survival, along with other prognosis factors. A multi-institutional retrospective study assessed CDH patient characteristics and survival rates by analyzing factors including the first cry, disease severity, birth weight, Apgar scores, oxygenation index (OI) and surgical closure. Among the CDH patients in the study, a positive first cry was linked to 100% survival, regardless of disease severity (p < 0.001). Notably, the presence of a positive first cry did not significantly affect survival rates in patients with worse prognostic factors, such as low birth weight (<2500 g), high CDH severity, low Apgar scores (1 min ≤ 4), high best OI within 24 h after birth (≥8), or those who underwent patch closure. Furthermore, no significant association was found between the first cry and the use of inhaled nitric oxide (iNO) or extracorporeal membrane oxygenation (ECMO). In conclusion, this study suggests that the first cry may not have a negative impact on the prognosis of CDH patients and could potentially have a positive effect.

Characterization of Suboptimal Responses to Fetoscopic Endoluminal Tracheal Occlusion in Congenital Diaphragmatic Hernia

Introduction: The aim of the study was to characterize the changes in fetal lung volume following fetoscopic endoluminal tracheal occlusion (FETO) that are associated with infant survival and need for extracorporeal membrane oxygenation (ECMO) in congenital diaphragmatic hernia (CDH). Methods: Fetuses with CDH who underwent FETO at a single institution were included. CDH cases were reclassified by MRI metrics [observed-to-expected total lung volume (O/E TLV) and percent liver herniation]. The percent changes of MRI metrics after FETO were calculated. ROC-derived cutoffs of these changes were derived to predict infant survival to discharge. Regression analyses were done to determine the association between these cutoffs with infant survival and ECMO need, adjusted for site of CDH, gestational age at delivery, fetal sex, and CDH severity. Results: Thirty CDH cases were included. ROC analysis demonstrated that post-FETO increases in O/E TLV had an area under the curve of 0.74 (p = 0.035) for the prediction of survival to hospital discharge; a cutoff of less than 10% was selected. Fetuses with a <10% post-FETO increase in O/E TLV had lower survival to hospital discharge [44.8% vs. 91.7%; p = 0.018] and higher ECMO use [61.1% vs. 16.7%; p = 0.026] compared to those with an O/E TLV increase ≥10%. Similar results were observed when the analyses were restricted to left-sided CDH cases. A post-FETO <10% increase in O/E TLV was independently associated with lower survival at hospital discharge (aOR: 0.073, 95% CI: 0.008–0.689; p = 0.022) and at 12 months of age (aOR: 0.091, 95% CI: 0.01–0.825; p = 0.036) as well as with higher ECMO use (aOR: 7.88, 95% CI: 1.31–47.04; p = 0.024). Conclusion: Fetuses with less than 10% increase in O/E TLV following the FETO procedure are at increased risk for requiring ECMO and for death in the postnatal period when adjusted for gestational age at delivery, CDH severity, and other confounders.

Surgical Risk Factors for Delayed Oral Feeding Autonomy in Patients with Left-Sided Congenital Diaphragmatic Hernia.

Congenital diaphragmatic hernia (CDH) is a rare disease associated with major nutritional and digestive morbidities. Oral feeding autonomy remains a major issue for the care and management of these patients. The aim of this study was to specify the perinatal risk factors of delayed oral feeding autonomy in patients treated for CDH. This monocentric cohort study included 138 patients with CDH. Eighty-four patients were analyzed after the exclusion of 54 patients (11 with delayed postnatal diagnosis, 5 with chromosomal anomaly, 9 with genetic syndrom, 13 with right-sided CDH, and 16 who died before discharge and before oral feeding autonomy was acquired). They were divided into two groups: oral feeding autonomy at initial hospital discharge (group 1, n = 51) and nutritional support at discharge (group 2, n = 33). Antenatal, postnatal, and perisurgical data were analyzed from birth until first hospital discharge. To remove biased or redundant factors related to CDH severity, statistical analysis was adjusted according to the need for a patch repair. After analysis and adjustment, delayed oral feeding autonomy was not related to observed/expected lung-to-head ratio (LHR o/e), intrathoracic liver and/or stomach position, or operative duration. After adjustment, prophylactic gastrostomy (OR adjusted: 16.3, IC 95%: 3.6-74.4) and surgical reoperation (OR adjusted: 5.1, IC 95% 1.1-23.7) remained significantly associated with delayed oral feeding autonomy. Delayed oral feeding autonomy occurred in more than one third of patients with CDH. Both prophylactic gastrostomy and surgical reoperation represent significant risk factors. Bowel obstruction might also impact oral feeding autonomy. Prophylactic gastrostomy seems to be a false "good idea" to prevent failure to thrive. This procedure should be indicated case per case. Bowel obstruction and all surgical reoperations represent decisive events that could impact oral feeding autonomy.

On–Extracorporeal Membrane Oxygenation Congenital Diaphragmatic Hernia Repair: Decreased Bleeding Complications and Improved Survival with Muscle Flap Repair Technique

INTRODUCTION: Prosthetic patches (patch) and muscle flaps (flap) are different techniques used for repair of congenital diaphragmatic hernia (CDH) with a large defect unamenable to primary closure. We hypothesized that the flap technique for CDH repair while on extracorporeal membrane oxygenation (on-ECMO) would have decreased bleeding complications compared with patch due to the hemostatic advantage of native tissue, and therefore also be associated with improved survival. METHODS: A single-center retrospective study of patients who underwent on-ECMO CDH repair between 2008 and 2021 was performed. Statistical significance was determined by Mann Whitney U, Fisher’s exact, chi-square, and Student’s unpaired t tests as appropriate (p < 0.05 significant). RESULTS: Forty-eight patients met criteria: 17 patch (35.4%) and 31 flap (64.6%). Characteristics between these 2 groups are summarized in Table 1. Reoperation for surgical bleeding was significantly decreased with flap repair compared with patch (35.5% vs 64.7%, respectively; p = 0.05). 48-hour postoperative blood product transfusion was also significantly decreased with flap repair (176.3 mL/kg vs 328.7 mL/kg patch; p = 0.027). Additionally, survival to hospital discharge was significantly increased in the flap repair group compared with the patch group (64.3% vs 27.8%, respectively; p = 0.033). Table 1. - Characteristics of Prosthetic Patch and Muscle Flap Repair for Congenital Diaphragmatic Hernia on Extracorporeal Membrane Oxygenation Variable Prosthetic patch repair (n = 17) Muscle flap repair (n = 31) p Value Left-sided defect 9 (52.9%) 26 (83.9%) 0.039* Gestational age at delivery (wk) 38 (37 - 39) 38 (37 - 38) 0.21† Birth weight (g) 2,842 ± 473.9 2,812 ± 430.4 0.82‡ Percent predicted lung volume 13.94 ± 5.1 14.99 ± 4.4 0.64‡ Observed-to-expected lung-to-head ratio 37.4 (31.1–42.0) 30 (21–42.5) 0.40† Days on ECMO 15 (8.5–28.5) 18 (12–25) 0.57† Values expressed as count (percentage of group), means ± standard deviation, or median (interquartile range). Statistical analysis by*Fisher’s exact test,†Mann Whitney U test, or‡Student’s unpaired t-test CONCLUSION: We conclude that the muscle flap technique for on-ECMO CDH repair is associated with decreased bleeding complications compared with prosthetic patch repair, which may in part be responsible for the improved survival seen in the flap repair group. Despite comparable CDH severity, these results support the flap repair technique as the safer method for on-ECMO CDH repair.

Antenatal Assessment of the Prognosis of Congenital Diaphragmatic Hernia: Ethical Considerations and Impact for the Management.

Congenital diaphragmatic hernia (CDH) is associated with abnormal pulmonary development, which is responsible for pulmonary hypoplasia with structural and functional abnormalities in pulmonary circulation, leading to the failure of the cardiorespiratory adaptation at birth. Despite improvement in treatment options and advances in neonatal care, mortality remains high, at close to 15 to 30%. Several risk factors of mortality and morbidities have been validated in fetuses with CDH. Antenatal assessment of lung volume is a reliable way to predict the severity of CDH. The two most commonly used measurements are the observed/expected lung to head ratio (LHRo/e) and the total pulmonary volume (TPV) on MRI. The estimation of total pulmonary volume (TPVo/e) by means of prenatal MRI remains the gold standard. In addition to LHR and TPV measurements, the position of the liver (up, in the thorax or down, in the abdomen) also plays a role in the prognostic evaluation. This prenatal prognostic evaluation can be used to select fetuses for antenatal surgery, consisting of fetoscopic endoluminal tracheal occlusion (FETO). The antenatal criteria of severe CDH with an ascended liver (LHRo/e or TPVo/e < 25%) are undoubtedly associated with a high risk of death or significant morbidity. However, despite the possibility of estimating the risk in antenatal care, it is difficult to determine what is in the child’s best interest, as there still are many uncertainties: (1) uncertainty about individual short-term prognosis; (2) uncertainty about long-term prognosis; and (3) uncertainty about the subsequent quality of life, especially when it is known that, with a similar degree of disability, a child’s quality of life varies from poor to good depending on multiple factors, including family support. Nevertheless, as the LHR decreases, the foreseeable “burden” becomes increasingly significant, and the expected benefit is increasingly unlikely. The legal and moral principle of the proportionality of medical procedures, as well as the prohibition of “unreasonable obstinacy” in all investigations or treatments undertaken, is necessary in these situations. However, the scientific and rational basis for assessing the long-term individual prognosis is limited to statistical data that do not adequately reflect individual risk. The risk of self-fulfilling prophecies should be kept in mind. The information given to parents must take this uncertainty into account when deciding on the treatment plan after birth.

Mediastinal Shift Angle in Fetal MRI Is Associated With Prognosis, Severity, and Cardiac Underdevelopment in Left Congenital Diaphragmatic Hernia.

Objective:Fetal MRI has played an essential role in the evaluation and management of congenital diaphragmatic hernia (CDH). We aimed to investigate whether the mediastinal shift angle (MSA) value was associated with the prognosis and the severity of left CDH and explore the relationship between the MSA value and fetal and neonatal cardiac structures and functions.MethodsFrom January 2012 to December 2020, the fetal MSA values of left CDH in our institution were retrospectively measured. Other prenatal parameters and clinical outcomes of them are collected. We also measured the fetal and postnatal echocardiography parameters to analyze linear correlation with MSA values.ResultsA total of 94 patients with left CDH were included. MSA was significantly higher in the deceased group than in the survived group [((38.3 ± 4.7)° vs. 32.3 ± 5.3)°, p < 0.001]. The MSA value of the high-risk defect group [CDH Study Group (CDHSG) C/D type] was significantly higher than that of the low-risk defect group [CDHSG A/B type; (36.0 ± 4.9)° vs. (30.1 ± 4.8)°, p < 0.001]. The AUC for severity was 0.766 (95% CI, 0.661–0.851, p < 0.0001) and the best cut-off value for MSA was 30.7°. Higher MSA correlates with decreased fetal Z-score of left ventricle (LV) width, the diameter of the mitral valve (MV), peak velocity of MV and tricuspid valve (TV), and neonatal LV end-diastolic diameter (LVEDD) and velocity of tricuspid regurgitation (TR; p < 0.05).ConclusionA high MSA value can effectively predict high-risk defects and high mortality of left CDH. The higher the MSA value, the worse the neonatal conditions, the respiratory and cardiovascular prognosis. The MSA values could reflect the level of left heart underdevelopment, including decreased dimensions and diastolic dysfunction of the left ventricle.

Neonatal rodent ventilation and clinical correlation in congenital diaphragmatic hernia.

Ventilator management is a critical part of managing congenital diaphragmatic hernia (CDH). We aimed to use a murine model and patient data to study CDH-associated differences in oxygenation, airway resistance, and pulmonary mechanics by disease severity. We used the nitrofen model of CDH. For control and CDH rodents, data were collected within the first hour of life. Oxygen saturations (SpO2 ) were collected using MouseOx, and large airway resistance and inspiratory capacities were collected using flexiVent. A single-center, retrospective review of term CDH infants from 2014 to 2020 was performed. Tidal volumes were collected every 6 h for the first 48 h of life or until the patient was taken off conventional ventilation. Newborns that were mechanically ventilated but had no pulmonary pathology were used as controls. CDH severity was defined using theCDH Study Group (CDHSG) classification system. Control rodents had a median SpO2 of 94% (IQR: 88%-98%); CDH pups had a median SpO2 of 27.9% (IQR: 22%-30%) (p < 0.01). CDH rodents had lower inspiratory capacity than controls (median:110 μl, IQR:70-170 vs. median:267 μl, IQR: 216-352;p < 0.01). CDH infants had a lower initial SpO2 than control infants. Overall, CDH infants had lower tidal volumes than control infants (median: 4.2 ml/kg, IQR: 3.3-5.0 vs. 5.4 ml/kg, IQR: 4.7-6.2;p = 0.03). Tidal volumes varied by CDHSG stage. Newborns with CDH have lower SpO2 and lower, CDHSG stage specific, tidal volumes than control infants. The nitrofen model of CDH reflects these differences. Rodent models may be useful in studying therapeutic ventilatory strategies for CDH infants.

Imaging Assessment of Prognostic Parameters in Cases of Isolated Congenital Diaphragmatic Hernia: Integrative Review.

Antenatal recognition of severe cases of congenital diaphragmatic hernia (CDH) by ultrasound (US) and magnetic resonance imaging (MRI) may aid decisions regarding the indication of fetal endoscopic tracheal occlusion. An integrative review was performed. Searches in MEDLINE and EMBASE used terms related to CDH, diagnosis, MRI, and US. The inclusion criteria were reviews and guidelines approaching US and MRI markers of severity of CDH published in English in the past 10 years. The search retrieved 712 studies, out of which 17 publications were included. The US parameters were stomach and liver positions, lung-to-head ratio (LHR), observed/expected LHR (o/e LHR), and quantitative lung index. The MRI parameters were total fetal lung volume (TFLV), observed/expected TFLV, relative fetal or percent predicted lung volumes, liver intrathoracic ratio, and modified McGoon index. None of the parameters was reported to be superior to the others. The most mentioned parameters were o/e LHR, LHR, liver position, o/e TFLV, and TFLV.

The fetal lamb model of congenital diaphragmatic hernia shows altered cerebral perfusion using contrast enhanced ultrasound

BackgroundNeurodevelopmental impairment is common in survivors of congenital diaphragmatic hernia (CDH). Altered cerebral perfusion in utero may contribute to abnormal brain development in CDH patients. Methods5 fetal lambs with surgical left-CDH and 5 controls underwent transuterine cranial Doppler and contrast enhanced ultrasound (CEUS). Global and regional perfusion metrics were obtained. Biometric and perfusion data were compared between groups via nonparametric Mann Whitney U test and Spearman's rank order correlation. ResultsNo significant differences in cerebral Doppler measurements were identified between groups. By CEUS, CDH animals demonstrated significantly decreased global brain perfusion and increased transit time. With focal regions-of-interest (ROIs), there was a tendency towards decreased perfusion in the central/thalamic region in CDH but not in the peripheral brain parenchyma. Transit time was significantly increased in both ROIs in CDH, whereas flux rate was decreased in the central/thalamic region but not the peripheral brain parenchyma. Biometric CDH severity was correlated to perfusion deficit. There was no difference in cardiomyocyte histology. ConclusionThe fetal lamb model of CDH shows altered cerebral perfusion as measured by CEUS, correlating to disease severity. This suggests a physiological abnormality in fetal cerebrovascular perfusion that may contribute to abnormal brain development and neurodevelopmental impairment in survivors.

Ретроспективный анализ результатов диагностики и лечения врожденной диафрагмальной грыжи плода, по данным перинатального консилиума

Study Objective: Analysis of the detection of congenital diaphragmatic hernia (CDH) in the fetus at the antenatal stage and perinatal outcomes of newborns with CDH in the Russian Federation in a sample of women who applied to the perinatal consultation of the National Medical Research Center for Obstetrics, Gynecology and Perinatology named after academician V.I. Kulakov. Study Design: Retrospective study. Materials and Methods. The study included 235 cases of CDH in fetuses and newborns were selected by the federal perinatal consultation of the National Medical Research Center for Obstetrics, Gynecology and Perinatology named after academician V.I. Kulakov. The following were retrospectively analyzed: medical and social characteristics of pregnant women, results of ultrasound and MRI examination of fetuses, delivery features and perinatal outcomes. Study Results. The number of women with fetal CDH in the perinatal consultation of the Center continues to grow every year. In 3/4 of the treated patients, fetal CDH was detected in the 2nd trimester of pregnancy, which exceeds the European level of detection. But against this background, an extremely low level of invasive prenatal diagnosis was noted. Detection using ultrasound and MRI of liver infection in the chest, including a group with a prognosis of high neonatal survival of 2.2–4 times. The perinatal mortality rate with CDH in the Centers detection is 43.4%. The most common method of delivery is the natural birth. The optimal term of delivery with the least increase in postnatal loss should be considered the period of 38–39 weeks of pregnancy. Forecast of survival of newborns. At the same time, the Apgar score does not have statistical indicators of differences in surviving and deceased newborns, regardless of the method of delivery, which is associated with a violation of child development. Conclusion. The level of detection of CDH and perinatal mortality of newborns is similar to the data of foreign perinatal centers that provide assistance to mothers and their newborns. Increasing the survival rate of newborns with CDH is possible with the development and implementation of an integrated system for diagnosing the severity of CDH, as this will open up the possibility of a personalized approach to the treatment of CDH. Keywords: congenital diaphragmatic hernia, perinatal mortality, perinatal consultation, pulmonary hypoplasia, cardiac compression.