Identification of protein biomarkers associated with congenital diaphragmatic hernia in human amniotic fluid.

Congenital diaphragmatic hernia (CDH) is a severe birth defect frequently associated with pulmonary hypoplasia, pulmonary hypertension, and heart failure. Since amniotic fluid comprises proteins of both fetal and maternal origin, its analysis could provide insights on mechanisms underlying CDH and provide biomarkers for early diagnosis, severity of pulmonary changes and treatment response. The study objective was to identify proteomic changes in amniotic fluid consistently associated with CDH. Amniotic fluid was obtained at term (37-39 weeks) from women with normal pregnancies (n = 5) or carrying fetuses with CDH (n = 5). After immuno-depletion of the highest abundance proteins, off-line fractionation and high-resolution tandem mass spectrometry were performed and quantitative differences between the proteomes of the groups were determined. Of 1036 proteins identified, 218 were differentially abundant. Bioinformatics analysis showed significant changes in GP6 signaling, in the MSP-RON signaling in macrophages pathway and in networks associated with cardiovascular system development and function, connective tissue disorders and dermatological conditions. Differences in selected proteins, namely pulmonary surfactant protein B, osteopontin, kallikrein 5 and galectin-3 were validated by orthogonal testing using ELISA in larger cohorts and showed statistically significant differences aiding in the diagnosis and prediction of CDH. The findings provide potential tools for clinical management of CDH.

Syndromic congenital diaphragmatic hernia: Current incidence and outcome. Analysis from the congenital diaphragmatic hernia study group registry.

BACKGROUND: The aim of this study was to describe the incidence of Congenital Diaphragmatic Hernia, CDH, associated with known or clinically suspected syndromes, and the postnatal outcomes from a large database for CDH. METHODS: Data from the multicenter, multinational database on infants with CDH (Congenital Diaphragmatic Hernia Study Group Registry) born from 1996 to 2020 were analyzed. Patients with known or suspected syndromes were grouped and outcome data were analyzed and compared to those without syndromic features. RESULTS: A total of 12,553 patients were entered in the registry during the study period, and 421 had reported known syndromes, representing 3.4% of all CDH cases in the registry. A total of 50 different associated syndromes were reported. In addition to those with clinically suspected genetic conditions, a total rate of genetic syndromes with CDH was 8.2%. The overall survival to discharge for syndromic CDH was 34% and for non-syndromic CDH was 76.7%. The most common were syndromes Fryns syndrome (19.7% of all syndromes, 17% survival), trisomy 18 or Edward syndrome (17.5%, 9% survival), trisomy 21 or Down syndrome (9%, 47% survival), trisomy 13 or Patau syndrome (6.7%, 14% survival), Cornelia de Lange syndrome (6.4% of all syndromes, 22% survival) and Pallister-Killian syndrome (5.5% of all syndromes, 39.1% survival). In addition, 379 cases had reported chromosomal anomalies and 233 cases had clinically suspected syndromes, based on two more dysmorphic features or malformations in addition to CDH, but without molecular diagnosis. The syndromic CDH group had lower birth weight and gestational age at birth and increased incidence of bilateral CDH (2.9%) and rates of non-repair (53%). The length of hospital stay was longer, and larger number of patients needed O2 at 30 days. Extracorporeal life support was used only in 15% of the cases. Those who underwent surgical repair had survival to discharge rates of 73%. CONCLUSION: Syndromic CDH is rare and only 3.4% of the reported cases of CDH have a known syndrome or association, but, if including patients with two dysmorphic features malformations, in addition to CDH, altogether as many as 8.2% have a diagnosed or suspected genetic condition. These children have with lower survival rates. Given higher rates of non-repair and decreased extracorporeal life support use, along with a high early mortality, decision-making regarding goals of care clearly influences outcomes. Survival varies depending on the genetic cause. Early genetic diagnosis is important and may influence the decision-making.

Impact of Repeat Extracorporeal Life Support on Mortality and Short-term In-hospital Morbidities in Neonates With Congenital Diaphragmatic Hernia.

OBJECTIVE: To evaluate the impact of repeat extracorporeal life support (ECLS) on survival and in-hospital outcomes in the congenital diaphragmatic hernia (CDH) neonates. BACKGROUND: Despite the widespread use of ECLS, investigations on multiple ECLS courses for CDH neonates are limited. METHODS: This is a retrospective cohort study of all ECLS-eligible CDH neonates enrolled in the Congenital Diaphragmatic Hernia Study Group registry between 1995 and 2019. CDH infants with estimated gestational age at birth <32 weeks and a birth weight <1.8 kg and/or with major cardiac or chromosomal anomalies were excluded. The primary outcomes were survival and morbidities during the index hospitalization. RESULTS: Of 10,089 ECLS-eligible CDH infants, 3025 (30%) received 1 ECLS course, and 160 (1.6%) received multiple courses. The overall survival rate for patients who underwent no ECLS, 1 ECLS course, and multicourse ECLS were 86.9±0.8%, 53.8±1.8%, and 43.1±7.7%, respectively. Overall ECLS survival rate is increased by 5.1±4.6% ( P =0.03) for CDH neonates treated at centers that conduct repeat ECLS compared with those that do not offer repeat ECLS. This suggests that there would be an overall survival benefit from increased use of multiple ECLS courses. Infants who did not receive ECLS support had the lowest morbidity risk, while survivors of multicourse ECLS had the highest rates of morbidities during the index hospitalization. CONCLUSIONS: Although survival is lower for repeat ECLS, the use of multiple ECLS courses has the potential to increase overall survival for CDH neonates. Increased use of repeat ECLS might be associated with improved survival. The potential survival advantage of repeat ECLS must be balanced against the increased risk of morbidities during the index hospitalization.

Hidden cardiovascular morbidity in children and young adults born with congenital diaphragmatic hernia: A population-based study.

INTRODUCTION: Congenital diaphragmatic (CDH) hernia is a rare congenital malformation with considerable mortality and morbidity in the neonatal period. The majority of the children today survive but little is known about long term cardiovascular morbidity. MATERIAL AND METHODS: This was a nationwide population-based prospective case-control study within a cohort of Swedish children with CDH, born 1982-2015. Five controls for each patient were randomly sampled from the population. The outcomes were the corresponding International Statistical Classification of Disease (ICD) codes for cardiovascular diagnoses according to ICD 9 and 10. RESULTS: There was an overrepresentation of cardiovascular diagnoses in the CDH group after one year of age compared to the control group, 8.0vs 0.5% (n = 53 versus n = 16). The risk of having a cardiovascular diagnosis in this CDH group was 15 times higher compared to the control group (HR 15.8, 95% CI: 9-27.6, p < 0.005). The diagnoses of cardiac arrhythmias and systemic hypertension were less common in the CDH group before the age of one year compared to the CDH group beyond the age of one year. Arrhythmia 3.7 vs 15.1%, systemic hypertension 3.7 vs 7.5%. CONCLUSION: CDH survivors have increased cardiovascular morbidity during childhood and young adulthood. This implies that structured follow up programs, covering cardiovascular morbidity, needs to be developed and should be offered in pediatric and adult care. Being born with CDH seems to be a risk factor for future cardiovascular diagnoses. LEVEL OF EVIDENCE: Level 3: Case-Control Study.

Extracorporeal Life Support Organization Guideline for Transport and Retrieval of Adult and Pediatric Patients with ECMO Support.

DISCLAIMER: This guideline for the preparation for and undertaking of transport and retrieval of patients on extracorporeal membrane oxygenation (ECMO) is intended for educational use to build the knowledge of physicians and other health professionals in assessing the conditions and managing the treatment of patients undergoing ECLS / ECMO and describe what are believed to be useful and safe practice for extracorporeal life support (ECLS, ECMO) but these are not necessarily consensus recommendations. The aim of clinical guidelines are to help clinicians to make informed decisions about their patients. However, adherence to a guideline does not guarantee a successful outcome. Ultimately, healthcare professionals must make their own treatment decisions about care on a case-by-case basis, after consultation with their patients, using their clinical judgement, knowledge and expertise. These guidelines do not take the place of physicians' and other health professionals' judgment in diagnosing and treatment of particular patients. These guidelines are not intended to and should not be interpreted as setting a standard of care or be deemed inclusive of all proper methods of care nor exclusive of other methods of care reasonably directed to obtaining the same results. The ultimate judgment must be made by the physician and other health professionals and the patient in light of all the circumstances presented by the individual patient, and the known variability and biological behavior of the clinical condition. These guidelines reflect the data at the time the guidelines were prepared; the results of subsequent studies or other information may cause revisions to the recommendations in these guidelines to be prudent to reflect new data, but ELSO is under no obligation to provide updates. In no event will ELSO be liable for any decision made or action taken in reliance upon the information provided through these guidelines.

Premature and Extracorporeal Life Support: Is it Time? A Systematic Review.

Early preterm birth < 34 gestational weeks (GA) and birth weight (BW) <2 kg are relative contraindications for extracorporeal membrane oxygenation (ECMO). However, with improved technology, ECMO is presently managed more safely and with decreasing complications. Thus, these relative contraindications may no longer apply. We performed a systematic review to evaluate the existing literature on ECMO in early and late (34-37 GA) prematurity focusing on survival to hospital discharge and the complication intracranial hemorrhage (ICH). Data sources: MEDLINE, PubMed, Web of Science, Embase, and the Cochrane Database. Only publications in the English language were evaluated. Of the 36 included studies, 23 were related to ECMO support for respiratory failure, 10 for cardiac causes, and four for congenital diaphragmatic hernia (CDH). Over the past decades, the frequency of ICH has declined (89-21%); survival has increased in both early prematurity (25-76%), and in CDH (33-75%), with outcome similar to late prematurity (48%). The study was limited by an inherent risk of bias from overlapping single-center and registry data. Both the risk of ICH and death have decreased in prematurely born treated with ECMO. We challenge the 34 week GA time limit for ECMO and propose an international task force to revise current guidelines. At present, gestational age < 34 weeks might no longer be considered a contraindication for ECMO in premature neonates.

Optimizing Contrast-Enhanced Thoracoabdominal CT in Patients During Extracorporeal Membrane Oxygenation.

RATIONALE AND OBJECTIVES: To evaluate the quality and value of contrast-enhanced (CE) chest- and abdominal computed tomography (CT) and CT angiography in neonates, children, and adults undergoing extracorporeal membrane oxygenation (ECMO) using a customized protocol for contrast delivery. MATERIALS AND METHODS: All patients admitted for CE thoracic- and/or abdominal CT while on ECMO were prospectively included in the study. A protocol for contrast delivery adapted for the type of ECMO circulation, cannulation sites, anatomy of interest, and desired contrast phase was applied. Clinical information, ECMO and CT technique, including contrast administration strategy, was noted for each patient. Two radiologists separately evaluated the quality of the scan. The value of the examination was decided in consensus with the referring ECMO physician. RESULTS: One hundred thirty CE thoracoabdominal scans were performed at 103 different occasions during the study time. Eighty-nine scans were performed during veno-arterial ECMO and 41 during veno-venous ECMO. In the majority, contrast was delivered to the oxygenator with preserved ECMO flow. A peripheral or central venous line with reduced flow was utilized in the remaining cases. Mean scan quality was graded 4.2 on a five-grade scale. In 56% of the examinations, the findings affected the immediate treatment of the patient. CONCLUSION: High-quality CT and CT angiography can be achieved in ECMO patients of different ages and clinical issues considering the type of ECMO circulation, ECMO cannulation sites, preferred contrast phase and anatomy of interest. CT diagnoses affect the treatment of the patient.

Re-ECMO for congenital diaphragmatic hernia: Is it worth the effort?

AIM: To evaluate the results in CDH patients subjected to a second course of ECMO at a single institution. MATERIAL AND METHODS: Retrospective review of medical charts of patients treated for CDH and ECMO in our center since 1990 to December 2018 was performed. For patients subjected to a second course of ECMO and who survived to hospital discharge charts from follow up visits were also reviewed. RESULTS AND DISCUSSION: From Jan 1990 until December 2018, 311 patients with CDH were treated in the department. 267 of these (86%) were discharged alive from the hospital and 81% (237/293) of the Swedish patients were alive by December 2018. 101 patients (32%) were subjected to ECMO treatment of whom 71 survived (70%). 22 patients underwent a second ECMO run and 13 of these survived to hospital discharge. Seven of the Swedish patients [19] were long-term survivors (37%). The vast majority was on V-A ECMO. CONCLUSIONS: It is possible to recannulate the right common carotid artery and internal jugular vein for a second course of venoarterial ECMO in CDH patients, who deteriorate severely after decannulation. Previous research has shown that long-term survivors subjected to ECMO twice reported similar frequencies of pulmonary, gastrointestinal, neurological and musculoskeletal sequelae as the long-term survivors, who needed ECMO support only once, and similar health-related quality of life. Regarding their psychosocial function, they scored within normal range in the behavioral, emotional and social scales domains. A second ECMO run may contribute to a higher survival and that the long-term morbidity among survivors is not more pronounced than among survivors after a single course of ECMO. It is therefore suggested that a second course of ECMO should be offered on the same indications as the first course. LEVEL OF EVIDENCE: III Case series.

Educational level and socioeconomic status in patients born with congenital diaphragmatic hernia: A population-based study.

BACKGROUND: Neurodevelopmental dysfunction is one of the most disabling outcomes for congenital diaphragmatic hernia (CDH) survivors and may have a long lasting impact in adult life. AIM: To evaluate to which extent being born with CDH has an impact on the educational level and socioeconomic status as a proxy for neurocognitive development. MATERIAL AND METHODS: Nationwide, population-based prospective study of newborn children in Sweden from 1982 to 2015. School grades, highest educational level and income were assessed through Swedish public registries. Children above 15 years of age with CDH were compared with randomly selected controls. RESULTS: A significantly higher number of cases (17% vs 10%) did achieve neither a school nor a university degree. Among those who achieved a degree there was no difference in the highest level of education. The qualification points in elementary school did not differ, but in high school female cases had significantly lower qualification points than female controls. There were no differences in individual disposable income between cases and controls. However, males had higher income compared to females. Prematurity and a long hospital stay had a negative impact on educational level. CONCLUSIONS: A higher proportion of children born with CDH compared to controls do not achieve a school degree. Among those who achieved a degree, the school achievements and educational level were similar to controls. Prematurity and a long hospital stay are risk factors for not achieving an educational degree. TYPE OF STUDY: Prognosis study (high-quality prospective cohort study with 99% of patients followed to the study end point). LEVEL OF EVIDENCE: Level I. I for a prognosis study - This is a high-quality, prospective cohort study with 99% of patients followed to the study end point.

Congenital diaphragmatic hernia and associated omphalocele: a study from the CDHSG registry.

BACKGROUND: Congenital Diaphragmatic Hernia (CDH) associated with Omphalocele is a rare condition, and only a few case reports are available in the literature. Both conditions are associated with some degree of pulmonary hypoplasia. We hypothesize that the combination of CDH with Omphalocele might be associated with poorer outcomes. AIM: The aim of this study was to describe the incidence of this association and postnatal outcomes from the largest database available for CDH. METHODS: Data from the multicenter, multinational database on infants with CDH (CDHSG Registry) born from 2007 to 2018 was analyzed. RESULTS: A total of 5730 entries were made into the registry during the study period. The incidence of Omphalocele associated with CDH was 0.63% (36 out of 5730). When comparing posterolateral Bochdalek hernias with Omphalocele (CDH + O) to CDH without Omphalocele (CDH-), CDH + O were born at significantly younger gestational ages. They were sicker directly after birth with significantly lower APGARs at all time points, but received ECMO significantly less often. The distribution of left vs right side or the defect size did not differ but CDH + O required patch in a significantly larger extent. CDH + O had surgical repair significantly later and had significantly higher rates of non-repairs and significantly lower survival rates. The morbidity was significantly higher with longer hospital stays and higher requirements for O2 at 30 DOL. DISCUSSION: CDH associated with Omphalocele is a rare but more severe condition with higher mortality and morbidity rates. Newborns with these combined conditions can be difficult to stabilize or might pose complicated management problems due to pulmonary hypertension and/or pulmonary hypoplasia. TYPE OF STUDY: Prognosis Study. LEVEL OF EVIDENCE: Level I.

Introducing the Loop for Circuit Access during Extracorporeal Membrane Oxygenation: Feasibility and Safety.

In extracorporeal membrane oxygenation (ECMO), blood is drained from the patient, and pumped through a membrane oxygenator/lung (ML) for gas exchange and then back to the patient. For monitoring blood gases, samples may be sampled downstream from the ML. This exposes the patient for embolization risk (air/clot) when the stopcocks are flushed. For safe sampling procedures, the Loop was introduced. It is a constant low-flow arteriovenous shunt (AVS) used preferably in venoarterial ECMO. It is composed of three different length and diameter three-way stopcocks connected to the circuit just downstream the ML with its return upstream the pump. It offers safe arterial blood sampling and a simultaneous access point to the venous side of the circuit. Since its introduction, no patient complications have been reported to be accounted for by the Loop. The Loop is an AVS permitting a safe access point for post membrane blood sampling and for injections in the venous pre-pump limb. It has a low cost and is easy to install and maintain. It may be used in any ECMO configuration.

Ready for Transfer to Adult Care? A Triadic Evaluation of Transition Readiness in Adolescents With Congenital Heart Disease and Their Parents.

Transfer to adult care for adolescents with chronic conditions ought to be determined by transition readiness. The aims of this study were (a) to describe the level of readiness for transition in adolescents with congenital heart disease, (b) to compare adolescents' assessment of transition readiness with their parents' assessments, and (c) to study potential correlates of transition readiness. A total of 157 triads of adolescents aged 14 to 18 years and their parents completed the Readiness for Transition Questionnaire. Adolescents scored higher on overall readiness than their parents. Multivariable analyses revealed that higher levels of adolescents' overall readiness were associated with a less threatening view of the illness, a higher level of empowerment, and with higher mothers' and fathers' overall readiness scores. Adolescents' responsibility scores were positively associated with age and parental adolescent responsibility scores. Parental involvement scores were negatively associated with adolescents' age and positively with the mothers' parental involvement scores. By using a triadic evaluation, the results of the present study significantly extend what is currently known about this population.

Long-term pulmonary function and quality of life in adults after extracorporeal membrane oxygenation for respiratory failure.

BACKGROUND: There is a significant long-term burden on survivors after acute respiratory distress syndrome, even 5 years after discharge. This is not well investigated in patients treated with extracorporeal membrane oxygenation. The objective of this study was to describe very-long-term (⩾3 years) disability in lung function and morphology, quality of life, mood disorders, walking capacity, and return to work status in extracorporeal membrane oxygenation survivors. METHODS: Single-center retrospective cohort study on long-term survivors treated with extracorporeal membrane oxygenation for respiratory failure between 1995 and 2010 at a tertiary referral center in Sweden. Eligible patients were approached, and those who consented were interviewed and investigated during a day at the hospital. RESULTS: A total of 38 patients were investigated with a median follow-up time of 9.0 years. Quality of life was reduced in several Short form 36 (SF-36) subscales and all domains of the St George's Respiratory Questionnaire, similar to previous studies in conventionally managed acute respiratory distress syndrome survivors. A reduced diffusion capacity of carbon monoxide was seen in 47% of patients, and some degree of residual lung parenchymal pathology was seen in 82%. Parenchymal pathology correlated with reductions in quality of life and diffusion capacity. Symptoms of anxiety and depression were seen in 22% and 14%, respectively. CONCLUSION: A significant long-term burden remains even 3-17 years after extracorporeal membrane oxygenation treatment, similar to conventionally managed acute respiratory distress syndrome survivors. Future prospective studies are needed to elucidate risk factors for these sequelae.

Differential hypoxemia during venoarterial extracorporeal membrane oxygenation.

Venoarterial extracorporeal membrane oxygenation, indicated for severe cardio-respiratory failure, may result in anatomic regional differences in oxygen saturation. This depends on cannulation, hemodynamic state, and severity of respiratory failure. Differential hypoxemia, often discrete, may cause clinical problems in peripheral femoro-femoral venoarterial extracorporeal membrane oxygenation, when the upper body is perfused with low saturated blood from the heart and the lower body with well-oxygenated extracorporeal membrane oxygenation blood. The key is to diagnose and manage fulminant differential hypoxemia, that is, a state that may develop where the upper body is deprived of oxygen. We summarize physiology, assessment of diagnosis, and management of fulminant differential hypoxemia during venoarterial extracorporeal membrane oxygenation. A possible solution is implantation of an additional jugular venous return cannula. In this article, we propose an even better solution, to drain the venous blood from the superior vena cava. Drainage from the superior vena cava provides superiority to venovenoarterial configuration in terms of physiological rationale, efficiency, safety, and simplicity in clinical circuit design.

Maternal Risk Factors and Perinatal Characteristics in Congenital Diaphragmatic Hernia: A Nationwide Population-Based Study.

BACKGROUND: The etiology of congenital diaphragmatic hernia (CDH) remains poorly understood. We hypothesize that environmental factors play an important role in the development of CDH. AIM: The objective of this study was to investigate associated maternal risk factors in pregnancies with CDH. MATERIAL AND METHODS: The study was a nationwide, population-based prospective case-control study consisting of a cohort of newborn children entered into the records of pregnant women receiving antenatal care in Sweden, registered in the Medical Birth Registry during the period from January 1, 1982 to December 31, 2015. The study outcome CDH and the different exposures were assessed through linkage to the Swedish National Patient Registry for both cases and mothers. RESULTS: A total of 972 cases of CDH were registered into one of the national registries in Sweden between 1982 and 2015. The incidence of neonates with CDH in Sweden from 1982 to 2015 was 3/10,000 live births. The mortality rate during the study period was 31%. Maternal age, ethnicity, parity, exposure to tobacco, BMI, IVF, previous history of spontaneous abortion or intrauterine fetal demise, and coexisting chronic diseases (urinary tract infection, chronic renal disease, pregestational diabetes, epilepsy, asthma, ulcerative colitis, inflammatory bowel disease, or systemic lupus erythematous) were not associated with an increased risk of CDH in the fetus. There was a significant association between maternal hypertension and the risk of the child being affected by CDH (OR 3.32, 95% CI 1.41-7.79, p = 0.01). No association was found between preeclampsia and CDH. CONCLUSIONS: Pregestational hypertension is associated with an increased risk of giving birth to a baby with CDH, but no association was observed in pregnancies developing preeclampsia and the occurrence of CDH.

Transport on extracorporeal membrane oxygenation for congenital diaphragmatic hernia: A unique center experience.

BACKGROUND: Support on Extracorporeal oxygenation membrane (ECMO) represents the last therapeutic option in the management of respiratory failure and pulmonary hypertension refractory to treatment in patients with congenital diaphragmatic hernia (CDH). AIM: The objective of this work was to present our experience of all the cases of CDH that we have transported on ECMO. MATERIAL AND METHODS: Medical records of patients, national and international, with CDH transported by our service on ECMO from 1997 to 2018 were reviewed. RESULTS: During 22 years, we performed 40 ECMO transports of newborns with CDH, 39 primary and one secondary. In 10% (4/40) we transferred patients from their primary hospital after the implantation of cannulae and commencement of ECMO to another center abroad owing to the lack of beds in our unit. Twenty (50%) of the transports were from a foreign country. Median transport distance was 560 (428-1381) km and the median transport time was 4.5 (4.2-6.3) h. The mode of transport was ground ambulance in 20%, helicopter in 10%, fixed wing aircraft in 62.5% and ground ambulance in Freight aircraft in 7.5%. In 40% of the transports, 20 complications occurred. In one of every four transports with complications, more than one event occurred. Most frequent complication was loss of tidal volumes (35%) and in 30% of the complications another patient related event was recorded. Equipment failure occurred in 20%, and climate problems and transport vehicle problems in 15%. No deaths occurred during transport. Venoarterial ECMO was used in 39 of the 40 cases. Survival to discharge was 87% for the entire period and long-term survival was 77%. CONCLUSIONS: Long and short distance interhospital transports of CDH patients on ECMO can be performed safely. Despite occurrence of adverse events, the risk of mortality is very low. The personnel involved must be highly competent in intensive care, physiology and physics of ECMO, cannulation, intensive care transport and air transport medicine. They must also be trained to recognize risk factors in these patients. LEVEL OF EVIDENCE: III Retrospective cohort study.

Quality of life and functional outcome in Swedish children with low anorectal malformations: a follow-up study.

PURPOSE: The aim was to investigate the quality of life and bowel function in children with low anorectal malformations (ARM). ADDITIONAL AIM: To evaluate the Swedish version the Hirschsprung's Disease/Anorectal Malformation Quality of life Questionnaire (HAQL). METHODS: Forty-four children and their parents were invited to complete the HAQL and the Bowel Function Score (BFS). Healthy children participated as controls and completed the HAQL. RESULTS: Seventeen children and 18 mothers completed the HAQL. The children reported impaired function in the physical symptom (PH) fecal continence (FC) and laxative diet (LD) domains compared to controls. Compared with their mothers, they reported impaired physical function and more symptoms in the emotional functioning (EMF) and PH domains. 27 families completed the BFS; 63% reported normal bowel function, 33% moderate outcome and one patient, comprising 4%, poor outcome. Evaluation of the HAQL, FC, EMF and PH domains showed no obvious conflicts. CONCLUSIONS: The children did not differ much regarding their QoL, even though they appeared to have impaired bowel function and worse emotional functioning compared to controls. The mothers underestimated their children's physical symptoms and overestimated their emotional functioning. Evaluated domains in the HAQL appear to work as intended, but the questionnaire needs further development.

Prenatally versus postnatally diagnosed congenital diaphragmatic hernia - Side, stage, and outcome.

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 < 0.0001). 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).

Parental uncertainty about transferring their adolescent with congenital heart disease to adult care.

AIMS: To study parent's levels of uncertainty related to the transfer from pediatric to adult care in adolescents with congenital heart disease (CHD) and to identify potentially correlating factors. BACKGROUND: Parents acknowledge that during transition they struggle with finding ways of feeling secure in handing over the responsibility and letting go of control. Well-prepared and informed parents who feel secure are most likely better skilled to support their adolescent and to hand over the responsibility. DESIGN: A cross-sectional study. METHODS: Overall, 351 parents were included (35% response rate). Parental uncertainty was assessed using a Linear Analogue Scale (0-100). Data were collected between January - August 2016. Potential correlates were assessed using the readiness for transition questionnaire and sociodemographic data. RESULTS: The mean parental uncertainty score was 42.5. Twenty-four percent of the parents had a very low level of uncertainty (score 0-10) and 7% had a very high level (score 91-100). Overall, 26% of the mothers and 36% of the fathers indicated that they had not started thinking of the transfer yet. The level of uncertainty was negatively associated with the level of perceived overall readiness. Adolescents' age, sex, CHD complexity, and parental age were not related to uncertainty. CONCLUSION: A wide range in the levels of uncertainty was found. Parents who were less involved in the care, or perceived their adolescent as readier for the transition, felt less uncertain. Still, thirty percent of the parents had not started to think about the transfer to adult care.

A Single-Center Experience of 900 Interhospital Transports on Extracorporeal Membrane Oxygenation.

BACKGROUND: The dawning of the extracorporeal membrane oxygenation (ECMO)-2 era, with the potential of decentralizing ECMO treatment, has stressed the need for research into the safety of ECMO transportations. The aim of this study was to (1) provide a comprehensive summary of transport arrangements and complications at a high-volume ECMO center, (2) determine predictors of severe complications occurring during transport, and (3) determine transport-related predictors of mortality. METHODS: This was a retrospective population-based observational cohort study of all interhospital ECMO transports performed by the Karolinska University Hospital between 1996 and 2017. Medical records, clinical notes, and original transport protocols were collected and reviewed. RESULTS: A total of 908 ECMO transports were performed. Neonatal and pediatric patients were more likely to be subjected to international transport, air transport, and longer transport distances and transport times. A severe complication occurred in 20% of transports and was significantly associated with venoarterial ECMO (p = 0.04) and fixed-wing transport (p = 0.01). Severe transport complications were not associated with increased mortality. Two patients passed away during transportation. CONCLUSIONS: Severe complications during ECMO transportation recurrently occurred but did not affect mortality. We conclude that interhospital ECMO transportation is safe, when conducted by an experienced center, and patients should be transported for treatment at a high-volume ECMO center in accordance with the hub-and-spoke model whenever feasible.