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.

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.

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.

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.

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.

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.

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.

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.

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 Cognitive Outcome and Brain Imaging in Adults After Extracorporeal Membrane Oxygenation.

OBJECTIVES: To investigate the presence of cognitive dysfunction and brain lesions in long-term survivors after treatment with extracorporeal membrane oxygenation for severe respiratory failure, and to see whether patients with prolonged hypoxemia were at increased risk. DESIGN: A single-center retrospective cohort study. SETTING: Tertiary referral center for extracorporeal membrane oxygenation in Sweden. PATIENTS: Long-term survivors treated between 1995 and July 2009. Seven patients from a previously published study investigated with a similar protocol were included. INTERVENTIONS: Brain imaging, neurocognitive testing, interview. MEASUREMENTS AND MAIN RESULTS: Thirty-eight patients (i.e., n = 31 + 7) were enrolled and investigated in median 9.0 years after discharge. Only memory tests were performed in 10 patients, mainly due to a lack of formal education necessary for the test results to be reliable. Median full-scale intelligence quotient, memory index, and executive index were 97, 101, and 104, respectively (normal, 100 ± 15). Cognitive function was not reduced in the group with prolonged hypoxemia. Brain imaging showed cerebrovascular lesions in 14 of 38 patients (37%), most commonly in the group treated with venoarterial extracorporeal membrane oxygenation (7/11, 64%). In this group, memory function and executive function were significantly reduced. CONCLUSIONS: Patients treated with extracorporeal membrane oxygenation for respiratory failure may have normal cognitive function years after treatment, if not affected by cerebrovascular lesions. Permissive hypoxemia was not correlated with long-term cognitive dysfunction in the present study. Further prospective studies with minimal loss to follow-up are direly needed to confirm our findings.

Health-related quality of life in children born with congenital diaphragmatic hernia.

PURPOSE: The aim of this study was to examine health-related quality of life (HRQoL) in children born with congenital diaphragmatic hernia (CDH). METHODS: Between 1993 and 2003, a total of 102 children born with CDH were treated at Astrid Lindgren Children's hospital in Stockholm. In 2012, long-term survivors (n = 77) were asked to participate in the present study, which resulted in a 46% (n = 35) response rate. The KIDSCREEN-52 questionnaire was used for measuring HRQoL and a detailed review of medical records was performed. RESULTS: The study participants did not differ from the non-participants in terms of prenatal diagnosis, gender, side of lesion, method of surgical repair, time to intubation, need for ECMO support, or way of discharge from the hospital. Children born with CDH considered themselves to have a good HRQoL, as good as healthy Swedish children. There were only a few significant HRQoL differences within the group of children with CDH, although several median scores in ECMO-treated patients were somewhat lower. Correlations between child and parent scores on HRQoL were low. CONCLUSIONS: Health-related quality of life in children born with CDH is good overall, however, a correlation between the severity of the malformation and HRQoL cannot be excluded.

Behavioral, emotional and social functioning in children born with congenital diaphragmatic hernia.

PURPOSE: The aim was to investigate social competence and behavioral and emotional problems in children and adolescents born with CDH. METHODS: All children born with CDH, treated in Stockholm 1990-2009, were invited to participate. After written consent, the Child Behavior Checklist or Adult Self-Report questionnaires were sent to participants. Of the 145 long-term survivors, 51% returned a completed questionnaire. Both the syndrome and competence scales were used and open-ended questions were analyzed with manifest content analysis. RESULTS: All parents of children aged 1.5-5 years and 90% of parents of children aged 6-18 years reported a normal range on the syndrome scale. Five parents indicated internalizing, but none externalizing behavior. All young adults achieved a normal score on the syndrome scale. Eighty-five percent had normal school achievement, 79% had normal social scores and 40% had normal activity levels. Significantly fewer boys (23%) were in the normal activity range compared with 67% of girls. CONCLUSIONS: The vast majority of all parents of children born with CDH scored no behavioral or emotional problems, furthermore, they reported normal social and school competence. However, the activity levels seemed to be reduced in children born with CDH.

Impact of Low Anorectal Malformation on Parenting Stress: A Mixed-Method Study.

The purpose of this study was to investigate parenting stress among parents of children with low ARM. STUDY AIMS: 1) Compare parenting stress among parents of children with low ARM, with parents of healthy children using questionnaires. 2) Identify subscales within the questionnaire which needed to be further explored. 3) Use semi-structured interviews with parents of children with low ARM, to explore parenting stress and to explain, expand and or support the quantitative findings. DESIGN AND METHODS: An explanatory sequential mixed methods design was used in this follow up study. The parents completed the Swedish Parenthood Stress Questionnaire (SPSQ), semi-structured interviews were conducted. RESULTS: Fifteen mothers and 13 fathers of children with low ARM age 8-18, returned completed questionnaires. A control group of 17 mothers and 6 fathers of healthy children age 8-18 that had visited the hospital for a minor procedure was recruited for comparison purposes. There were no significant differences found between index group and controls except in the subscale Incompetence, where parents of children with low ARM reported lower levels of stress compared to controls. Nine semi-structured interviews were conducted with parents of children with low ARM. Qualitative content analysis was used and revealed three themes - Communication between parents, Expectations of parenthood, and Challenges concerning parenthood. CONCLUSIONS: Parents of children with low ARM did not report high levels of stress. When interviewed, they told about earlier experiences of emotional stress, feelings of guilt, and chaos at the time the child was born and during infancy.

Long-Term Survival in Adults Treated With Extracorporeal Membrane Oxygenation for Respiratory Failure and Sepsis.

OBJECTIVE: The use of extracorporeal membrane oxygenation in adults with respiratory failure and sepsis is steadily increasing, but the knowledge on long-term survival in this group is scarce. The aim of the present study was to investigate the 5-year survival rates and causes of late death in this group of patients. DESIGN: Single-center retrospective cohort study. SETTING: Karolinska University Hospital, Stockholm, Sweden. PATIENTS: Adult patients treated with extracorporeal membrane oxygenation for respiratory failure and sepsis between the service being established for adults in 1995 and December 2013. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Survival status was attained from a national Causes of Death registry. Minimal patient background data, along with data on survival and causes of death were collected. Survival rates were calculated using the Kaplan-Meier method. Of 255 subjects, 64% survived to discharge. The median follow-up time in survivors was 4.4 years. There was a high mortality rate within the first months after discharge. In the group of patients who survived the first 90 days after treatment, the 5-year survival rate was 87% and was particularly beneficial in patients treated for infectious diseases (88-100%). Late deaths were seen in most diagnostic groups, but the Kaplan-Meier curves flattened out over time. CONCLUSIONS: Extracorporeal membrane oxygenation treatment in adult patients with respiratory failure and sepsis can be lifesaving in appropriately selected patients. For patients who survive the first months after extracorporeal membrane oxygenation treatment, long-term survival seems good, especially in patients treated for infections.

Neurologic Injury in Adults Supported With Veno-Venous Extracorporeal Membrane Oxygenation for Respiratory Failure: Findings From the Extracorporeal Life Support Organization Database.

OBJECTIVES: To assess in-hospital neurologic (CNS) complications in adult patients undergoing veno-venous extracorporeal membrane oxygenation for respiratory failure. DESIGN: Retrospective analysis of the Extracorporeal Life Support Organization's data registry. SETTING: Data reported to Extracorporeal Life Support Organization from 350 international extracorporeal membrane oxygenation centers during 1992-2015. PATIENTS: Adults (≥ 18 yr old) supported with veno-venous extracorporeal membrane oxygenation for respiratory failure. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We included 4,988 adults supported with veno-venous extracorporeal membrane oxygenation for respiratory failure. Neurologic injury was defined as brain death, seizures, stroke, and intracranial hemorrhage occurring during extracorporeal membrane oxygenation support. We used multivariable logistic regression to explore patient and extracorporeal membrane oxygenation factors associated with neurologic injury. Median age of the study cohort was 46 (interquartile range, 32-58). Four hundred twenty-six neurologic complications were reported in 356 patients (7.1%), and included 181 intracranial hemorrhage (42.5%), 100 brain deaths (23.5%), 85 stroke (19.9%), and 60 seizure events (14.1%). In-hospital mortality was significantly higher for those with CNS complications (75.8% vs 37.8%; p < 0.001) and varied by type of CNS injury; mortality was 79.6% in patients with intracranial hemorrhage, 68.2% in patients with stroke, and 50% in patients with seizures. Pre-extracorporeal membrane oxygenation cardiac arrest, continuous veno-venous hemofiltration, and hyperbilirubinemia during extracorporeal membrane oxygenation were associated with increased odds of neurologic injury. CONCLUSIONS: Approximately 7% of adults supported with veno-venous extracorporeal membrane oxygenation for respiratory failure had neurologic injury. Intracranial hemorrhage was the most frequent type, and survival for patients with neurologic injury was poor. Future investigations should evaluate anticoagulation management as well as brain/extracorporeal membrane oxygenation interaction to reduce these life-threatening events.

Adolescents with congenital heart disease: their opinions about the preparation for transfer to adult care.

The aim of the study was to explore what adolescents with congenital heart disease (CHD) view as important in the preparation for the transfer to adult care. We performed interviews in four focus groups with adolescents (14-18 years old) at four university hospitals in Sweden. Data was analysed using qualitative content analysis. The analysis revealed one main category; Becoming a manager of the condition and four subcategories; Sufficient knowledge about the health, Be a participant in the care, Parental support, and Communicate with others about the health. The adolescents' ages differentiated the discussion in the groups. The older adolescents seemed to have more interest in transition planning, information and transfer. The younger described more frustrations about communication and handling the disease. CONCLUSION: To become a manager of the CHD in daily life, the adolescents want disease specific knowledge, which should be communicated in a developmentally appropriate way. Adolescents want to participate and be involved in the transition process. They need support and guidance in how to communicate their CHD. Parental support is fundamental but it change over time. Moreover, peer-support is becoming more significant during the transition process. What is Known: • Transition during adolescence and transfer to adult care for adolescents with CHD is complex, and there is a shift in roles. • Adolescents often have poor knowledge and understanding about their heart condition and the consequences. What is New: • Adolescents call for disease specific information regarding health issues of importance for them in daily life. • Communicating the disease with other is a challenge- peer support from other adolescents with CHD could be a facilitator.

Long-Term Survival and Causes of Late Death in Children Treated With Extracorporeal Membrane Oxygenation.

OBJECTIVE: Extracorporeal membrane oxygenation has been used in patients with severe circulatory or respiratory failure since the 1970s, but the knowledge on long-term survival in this group is scarce. The aim of the present study was to investigate the 10-year survival rates and causes of late death in children treated with extracorporeal membrane oxygenation. DESIGN: Single-center, retrospective cohort study. SETTING: Tertiary referral center for extracorporeal life support. PATIENTS: Neonatal and pediatric patients treated with extracorporeal membrane oxygenation from 1987 to December 2013. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Survival status was obtained from the national Causes of Death registry. Patient background data along with data on survival and causes of death were collected. Survival rates were calculated using the Kaplan-Meier method. Of 400 subjects, 76% survived to discharge. The median follow-up time in survivors was 7.2 years. There was a high mortality rate within the first months after discharge. In the group of patients who survived the first 90 days after treatment, the 10-year survival rates were 93% in neonates and 89% in pediatric patients and were particularly beneficial in patients whose indication for extracorporeal membrane oxygenation was meconium aspiration syndrome, trauma, or infectious diseases. Late deaths were seen in some diagnostic groups, but the Kaplan-Meier curves plateaued over time. CONCLUSIONS: Children who survive the first months after treatment with extracorporeal membrane oxygenation have a high long-term survival rate. The prognosis is especially favorable in patients with reversible conditions.

Adverse Events during Inter-Hospital Transports on Extracorporeal Membrane Oxygenation.

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) may be a lifesaving rescue therapy in refractory, severe respiratory, and/or circulatory failure. To provide the best cost efficiency to the population served and patient outcome, ECMO therapy should be provided by specialized high volume ECMO centers. This requires dedicated transport teams to organize and perform these complex transports. Concerning adverse events and complications during these transfers, only a minimal amount of data has been published. METHODS: To shed light on this matter, all medical transport records from transports on ECMO between January 2010 and June 2016 were analyzed. The data was classified in constituent groups and categorized to risk groups. RESULTS: During the study period, 536 transports on ECMO were performed. The transport records could be identified in 514 of these cases (95.9%). In 163 (31.7%) transports 206 adverse events occurred. In 34 transports two or more adverse events passed on the same trip. No deaths occurred during transport. Sixty-five percent (134) of the complications were Patient related; the most prominent was loss of tidal volume with or without fluid flooding of the lung (n = 57, 43%). Lack of control of equipment was the most common Staff related flaw. Causes due to Equipment/technical (n = 30) could be traced to 14.6% of the events. Vehicle/transportation related complications were reported from 26 transfers, a sub-group in which 50% of the reports concerned malfunction of Ambulance utility/electrical, or Wrong ambulance size at hospital or airport. CONCLUSIONS: If transporting on ECMO high-risk or sudden threat-of-life situations are inevitable and have to be dealt with immediately, sometimes within seconds. A well-trained staff and an experienced high-volume organization are recommended. Key words: extra corporeal membrane oxygenation; ECMO, transport; adverse event; complication.

Venous Cannula Positioning in Arterial Deoxygenation During Veno-Arterial Extracorporeal Membrane Oxygenation-A Simulation Study and Case Report.

Venoarterial extracorporeal membrane oxygenation (VA-ECMO) is indicated in reversible life-threatening circulatory failure with or without respiratory failure. Arterial desaturation in the upper body is frequently seen in patients with peripheral arterial cannulation and severe respiratory failure. The importance of venous cannula positioning was explored in a computer simulation model and a clinical case was described. A closed-loop real-time simulation model has been developed including vascular segments, the heart with valves and pericardium. ECMO was simulated with a fixed flow pump and a selection of clinically relevant venous cannulation sites. A clinical case with no tidal volumes due to pneumonia and an arterial saturation of below 60% in the right hand despite VA-ECMO flow of 4 L/min was described. The case was compared with simulation data. Changing the venous cannulation site from the inferior to the superior caval vein increased arterial saturation in the right arm from below 60% to above 80% in the patient and from 64 to 81% in the simulation model without changing ECMO flow. The patient survived, was extubated and showed no signs of hypoxic damage. We conclude that venous drainage from the superior caval vein improves upper body arterial saturation during veno-arterial ECMO as compared with drainage solely from the inferior caval vein in patients with respiratory failure. The results from the simulation model are in agreement with the clinical scenario.

Does permissive hypoxaemia during extracorporeal membrane oxygenation cause long-term neurological impairment?: A study in patients with H1N1-induced severe respiratory failure.

BACKGROUND: The Extracorporeal Life Support Organisation accepts permissive hypoxaemia in adult patients during extracorporeal membrane oxygenation (ECMO). The neurological long-term outcome of this approach has not yet been studied. OBJECTIVES: We investigated the prevalence of brain lesions and cognitive dysfunction in survivors from the Influenza A/H1N1 2009 pandemic treated with permissive hypoxaemia during ECMO for severe acute respiratory distress syndrome (ARDS). Our hypothesis was that this method is reasonable if tissue hypoxia is avoided. DESIGN: Long-term follow-up study after ECMO. SETTING: Karolinska University Hospital, Sweden, from October 2012 to July 2013. PATIENTS: Seven patients treated with ECMO for severe influenza A/H1N1-induced ARDS were studied 3.2 years after treatment. Blood lactate concentrations were used as a surrogate for tissue oxygenation. INTERVENTIONS: Neurocognitive outcome was studied with standardised cognitive tests and MRI of the brain. MAIN OUTCOME MEASURES: Cognitive functioning and hypoxic brain lesions after permissive hypoxaemia during ECMO. The observation period was the first 10 days of ECMO or the entire treatment period if shorter than 10 days. RESULTS: Eleven of 13 patients were still alive 3 years after ECMO. We were able to contact seven of these patients (mean age 31 years), who all agreed to participate in this study. Mean ±â€ŠSD peripherally measured arterial saturation during the observation period was 79 ±â€Š10%. Full-scale Intelligence Quotient was within one standard deviation or above from the mean of a healthy population in five patients, and was 1.5 SD below the mean in one patient. In one other patient, it could not be determined because of a lack of formal education. Memory functioning was normal in all patients. MRI showed no changes related to cerebral hypoxia. CONCLUSIONS: Permissive hypoxaemia during ECMO might not negatively affect long-term cognitive outcome if adequate organ perfusion is maintained. TRIAL REGISTRATION: at Clinicaltrials.gov NCT01763060.