Fetal MRI assessment of mediastinal shift angle in isolated left congenital diaphragmatic hernia: A new postnatal survival predictive tool?

OBJECTIVE: To quantify the mediastinal shift angle (MSA) in fetuses with isolated left congenital diaphragmatic hernia (CDH) by magnetic resonance imaging and evaluate survival. METHOD: Fetuses from singleton pregnancies with isolated left CDH were matched for gestational age with controls without thoracic malformations. For all fetuses the MSA was determined by two operators and inter-operator variability and differences between cases and controls were investigated. For all cases total fetal lung volume (TFLV) was calculated and the correlation between MSA and TFLV was assessed, and its predictive value towards survival was determined. RESULTS: Thirty-four fetuses were included as cases and 42 as controls. The mean gestational age for assessment of CDH fetuses was 32 weeks (range 27-38). Twenty-four fetuses survived until discharge and 10 did not. There was an excellent inter-operator reliability for measuring the MSA and a significant difference between MSA in cases and controls. There was an inverse correlation between MSA values and survival, a correlation between TFLV and survival and an inverse correlation between MSA and TFLV. The area under the ROC curve for MSA in predicting survival was 0.931 (95% CI 0.851-1.000). CONCLUSION: The MSA measured late in gestation correlates with postnatal survival in patients with isolated left CDH.

Timing of Prenatal Magnetic Resonance Imaging in the Assessment of Congenital Diaphragmatic Hernia.

INTRODUCTION: Fetal magnetic resonance imaging (MRI) has been used to stratify severity of congenital diaphragmatic hernia (CDH) after ultrasound diagnosis. The purpose of this study was to determine if timing of MRI influenced prediction of severity of outcome in CDH. METHODS: A single institution retrospective review of all CDH referred to our institution from February 2004 to May 2017 was performed. Patients were included if they underwent at least 2 fetal MRIs prior to delivery. Prenatal MRI indices including observed-to-expected total fetal lung volume (o/e TFLV) were evaluated. Indices were categorized by trimester, either 2nd (20-27 weeks gestation) or 3rd trimester (>28 weeks gestation) and further analyzed for outcome predictability. Primary outcomes were survival, extracorporeal membrane oxygenation (ECMO), and pulmonary hypertension (PAH). Student t test and logistic and linear regression were used for data analyses. RESULTS: Of 256 fetuses evaluated for CDH, 197 were further characterized by MRI with 57 having both an MRI in the 2nd and 3rd trimesters. There was an average of 9.95 weeks (±4.3) between the 1st and 2nd MRI. Second trimester o/e TFLV was the only independent predictor of survival by logistic regression (OR 0.890, p < 0.01). Third trimester MRI derived lung volumes were associated with, and independent predictors of, severity of PAH and need for ECMO. Interval TFLV growth was a strong predictor of PAH postnatally (OR 0.361, p < 0.01). Overall cohort survival was 79%. CONCLUSION: Accuracy of MRI lung volumes to predict outcomes is dependent on the -gestational age at the time of exam. While MRI lung volumes at either the 2nd or 3rd trimester are predictive of morbidity, 2nd trimester lung volumes strongly correlated with mortality.

Ultrasonographic assessment of mediastinal shift angle (MSA) in isolated left congenital diaphragmatic hernia for the prediction of postnatal survival.

Objectives: To quantify mediastinal shift in isolated congenital diaphragmatic hernia (CDH), by the introduction of a new ultrasonographic (US) marker, defined as mediastinal shift angle (MSA) and to evaluate its ability in predicting postnatal survival at discharge.Methods: Twenty-four consecutive fetuses from singleton pregnancies with isolated left-sided CDH were included in the study group and then subdivided into group A (16 survivors) and group B (8 nonsurvivors). The study group was matched with a control group of 95 fetuses from singleton pregnancies free from structural and/or chromosomal anomalies. On the same US stored images commonly used for lung-to-head ratio (LHR) measurement, a landmark line was drawn from a point on the posterior face of the vertebral body, splitting it into two equal parts, to the mid-posterior surface of the sternum. Another landmark line was then traced from the same point of the vertebral body to touch tangentially the lateral wall of the right atrium. The angle between these two lines was used to quantify mediastinal shift and called "mediastinal shift angle" (MSA).Results: Median MSA was significantly different between group A (34.3° range 29.3-45.9°) and group B (42.7° range 34.1-58.9°) (p < .001) and between study group as a whole and the control group (19° range 13.8-25.9°) (p < .001). Statistical analysis confirmed an inverse correlation between MSA values and survival (p = .004). The best cutoff value for MSA was 43.7°, which demonstrated the highest discriminatory power (sensitivity 63%; specificity 93.75%).Conclusions: In fetuses with isolated CDH, the mediastinal shift may be quantified using mediastinal shift angle (MSA) and this US marker, similarly to the widely accepted and used US prenatal prognostic indicators (LHR and O/E LHR), seems to reliably predict survival.

Risk-stratification enables accurate single-center outcomes assessment in congenital diaphragmatic hernia (CDH).

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.

Clinical cardiac assessment in newborns with prenatally diagnosed intrathoracic masses.

BACKGROUND: Congenital space-occupying thoracic malformations and diaphragmatic hernia have in common pulmonary hypoplasia. Our study aims to assess cardiac involvement during post-natal adaptation. METHODS: A retrospective study was carried out on newborns with prenatally diagnosed intrathoracic mass. Gathering for respiratory distress syndrome (RDS), 35 neonates were compared for clinical course, cardiovascular enzymes, ECG, and ultrasound. RESULTS: The analysis revealed a high left heart defect rate in patients with severe RDS, without being influenced by the laterality. Ultrasound or laboratory assessment did not detect altered cardiac dimension or cardiomyopathy. Solely ECG signs of right ventricular strain were found. Increased QT-dispersion, T-wave and cardiac variability alterations in the first hours were all expression of non-specific cardiac repolarization disorders but predict worse outcome. CONCLUSIONS: Although RDS is the predominant symptom, slight cardiac dysfunctions should be recognized for prompt treatment. Conventional examinations for postnatal adaption should be integrated by complementary investigations.

Utilization patterns of extracorporeal membrane oxygenation in neonates in the United States 1997-2012.

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) remains one of the most intensive therapies for newborns in the United States. However, there is limited information on resource utilization for neonates receiving ECMO. METHODS: We conducted a retrospective data analysis of the Kids' Inpatient Database from 1997 to 2012. Bivariate and multivariate analysis was completed to identify predictors of LOS, hospital costs and mortality. Cardiac and non-cardiac diagnoses of neonates receiving ECMO were also included in the bivariate and multivariable analysis. RESULTS: Of the 5151 ECMO cases, survival to discharge was 62%. 22% had a principal cardiac diagnosis. After adjusting for covariates, increased mortality was associated with treatment in the midwest compared to the northeast region (aOR=2.0, p<0.01) and decreased among neonates with a non-cardiac diagnosis (aOR=0.4, p<0.01). Living in midwest was associated with longer LOS by 13days and increased hospital costs by 63,000 dollars (p<0.01). When stratified by non-cardiac diagnoses, infants with a diagnosis of congenital diaphragmatic hernia was associated with increased mortality (2.3, p<0.01) and longer LOS (25, p<0.01) and increased costs (11,100, p<0.01). CONCLUSION: Neonates who received ECMO in certain regions of the United States were associated with poorer survival outcomes as well as increased LOS and hospital costs. TYPE OF STUDY: Retrospective study. LEVEL OF EVIDENCE: Level III.

Antenatal assessment of liver position, rather than lung-to-head ratio (LHR) or observed/expected LHR, is predictive of outcome in fetuses with isolated left-sided congenital diaphragmatic hernia.

OBJECTIVES: Respiratory morbidity in congenital diaphragmatic hernia (CDH) is associated with high mortality and adverse outcome. Accurate prenatal diagnosis is essential for prognosis and potential treatment in utero. The aim was to evaluate the prenatal ultrasound findings in assessing the respiratory prognosis in fetuses with isolated left-sided CDH. METHODS: We retrospectively analyzed the medical records of 59 prenatally diagnosed left-sided CDH cases managed at a tertiary perinatal center. RESULTS: Survival rate in the study group was 73% (43/59). We found no statistically significant relationship between survival and the presence of polyhydramnios, gestational age at diagnosis, lung-to-head ratio (LHR) and observed/expected LHR (O/E LHR) values, gestational age at birth and birth weight. Intrathoracic liver herniation was a statistically significant parameter adversely affecting survival (37.2% in survivors, 68.8% in non-survivors, p = 0.031) and logistic regression confirmed this relationship. The presence of pneumothorax and severe pulmonary hypertension were significantly associated with mortality (82% non-survivors versus 15% in survivors, p = 0.0001). CONCLUSION: Intrathoracic liver herniation seems to be a reliable parameter in the prediction of survival and neonatal respiratory morbidity in fetuses with isolated left-sided CDH. In contrast, we found no significant correlation between perinatal outcome and LHR, O/E LHR values, birth weight and gestational age.

Predictive outcome indexes in neonatal Congenital Diaphragmatic Hernia.

OBJECTIVE: We examined the reliability of the main prenatal and postnatal prognosis-related indexes that can be used to evaluate congenital diaphragmatic hernia (CDH) outcome. METHODS: Seventy-seven neonates with CDH were analyzed according to CDH prognosis-related factors, divided into prenatal findings, postnatal clinical values and postnatal predictive outcome scores applied at birth and within the first 12-24 h. The data are compared between two groups: survivors and non-survivors. RESULTS: During prenatal age, major associated anomalies, intrathoracic stomach, diagnosis prior to 25 weeks of gestational age and lung-to-head ratio < 0.6 were statistically significant, demonstrating their greater incidence in non-survivors. The majority of postnatal values at PICU admission were found to be reliable in identifying the CDH outcome: paO2/FiO2, oxygenation index, alveolar-arterial-O2 gradient, arterial-alveolar-O2 tension ratio, pH, mean blood pressure, body temperature. All the postnatal predictive outcome scores (Apgar 1' and 5', CDH-Study-Group equation, Score for Neonatal-Acute-Physiology II, SNAP-Perinatal-Extension II, Pediatric Risk of Mortality III and Wilford-Hall/Santa-Rosa formula) were statistically significant with more favorable values for prognosis in the survivors group. CONCLUSION: The chances of predicting CDH outcome are fairly high. During prenatal age, only a few findings may be obtained. Conversely, many postnatal indexes and scores can reliably predict such outcome.