| 1. |
- Cochius-den Otter, S, et al.
(author)
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The CoDiNOS trial protocol: an international randomised controlled trial of intravenous sildenafil versus inhaled nitric oxide for the treatment of pulmonary hypertension in neonates with congenital diaphragmatic hernia
- 2019
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In: BMJ open. - : BMJ. - 2044-6055. ; 9:11, s. e032122-
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Journal article (peer-reviewed)abstract
- Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm that impairs normal lung development, causing pulmonary hypertension (PH). PH in CDH newborns is the main determinant for morbidity and mortality. Different therapies are still mainly based on ‘trial and error’. Inhaled nitric oxide (iNO) is often the drug of first choice. However, iNO does not seem to improve mortality. Intravenous sildenafil has reduced mortality in newborns with PH without CDH, but prospective data in CDH patients are lacking.Methods and analysisIn an open label, multicentre, international randomised controlled trial in Europe, Canada and Australia, 330 newborns with CDH and PH are recruited over a 4-year period (2018–2022). Patients are randomised for intravenous sildenafil or iNO. Sildenafil is given in a loading dose of 0.4 mg/kg in 3 hours; followed by continuous infusion of 1.6 mg/kg/day, iNO is dosed at 20 ppm. Primary outcome is absence of PH on day 14 without pulmonary vasodilator therapy and/or absence of death within the first 28 days of life. Secondary outcome measures include clinical and echocardiographic markers of PH in the first year of life. We hypothesise that sildenafil gives a 25% reduction in the primary outcome from 68% to 48% on day 14, for which a sample size of 330 patients is needed. An intention-to-treat analysis will be performed. A p-value (two-sided) <0.05 is considered significant in all analyses.Ethics and disseminationEthics approval has been granted by the ethics committee in Rotterdam (MEC-2017-324) and the central Committee on Research Involving Human Subjects (NL60229.078.17) in the Netherlands. The principles of the Declaration of Helsinki, the Medical Research Involving Human Subjects Act and the national rules and regulations on personal data protection will be used. Parental informed consent will be obtained.Trial registration numberNTR6982; Pre-results.
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| 2. |
- Mesas-Burgos, C, et al.
(author)
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Lung morphology after late fetal tracheal ligation in rats
- 2006
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In: European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie. - : Georg Thieme Verlag KG. - 0939-7248. ; 16:3, s. 160-165
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Journal article (peer-reviewed)
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| 3. |
- Flam, B, et al.
(author)
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Authors' Response
- 2015
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In: Journal of intensive care medicine. - : SAGE Publications. - 1525-1489 .- 0885-0666. ; 52:1, s. 493-4
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Journal article (other academic/artistic)
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| 4. |
- Flam, B, et al.
(author)
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Pheochromocytoma-Induced Inverted Takotsubo-Like Cardiomyopathy Leading to Cardiogenic Shock Successfully Treated With Extracorporeal Membrane Oxygenation
- 2015
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In: Journal of intensive care medicine. - : SAGE Publications. - 1525-1489 .- 0885-0666. ; 30:6, s. 365-372
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Journal article (peer-reviewed)abstract
- Pheochromocytoma classically displays a variety of rather benign symptoms, such as headache, palpitations, and sweating, although severe cardiac manifestations have been described. We report a case of pheochromocytoma-induced inverted takotsubo-like cardiomyopathy leading to shock and cardiac arrest successfully treated with extracorporeal membrane oxygenation (ECMO) as a bridge to pharmacological therapy and curative adrenalectomy. A previously healthy 46-year-old woman presented to the emergency department with abdominal pain, dyspnea, nausea, and vomiting. Clinical evaluation revealed cardiorespiratory failure with hypoxia and severe metabolic acidosis. Computed tomography (CT) scan showed pulmonary edema and a left adrenal mass. Transthoracic echocardiography (TTE) displayed severe left ventricular dysfunction with inverted takotsubo contractile pattern. Despite mechanical ventilation and inotropic and vasopressor support, asystolic cardiac arrest ensued. The patient was resuscitated using manual chest compressions followed by venoarterial ECMO. Repeated TTEs demonstrated resolution of the cardiomyopathy within a few days. Laboratory results indicated transient renal and hepatic dysfunction, and CT scan of the brain displayed occipital infarctions. Biochemical testing and radionuclide scintigraphy confirmed a pheochromocytoma. Pharmacological adrenergic blockade was instituted prior to delayed adrenalectomy after which the diagnosis was histopathologically verified. The patient recovered after rehabilitation. We conclude that pheochromocytoma should be considered in patients presenting with unexplained cardiovascular compromise, especially if they display (inverted) takotsubo contractile pattern. Timely, adequate management might involve ECMO as a bridge to pharmacological therapy and curative surgery.
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| 7. |
- Holzgraefe, B., et al.
(author)
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Extracorporeal membrane oxygenation for pandemic H1N1 2009 respiratory failure
- 2010
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In: Minerva Anestesiologica. - 0375-9393 .- 1827-1596. ; 76:12, s. 1043-1051
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Journal article (peer-reviewed)abstract
- BACKGROUND: Severe respiratory failure related to infection with the pandemic influenza A/H1N1 2009 virus is uncommon but possibly life-threatening. If, in spite of maximal conventional critical care, the patient's condition deteriorates, extracorporeal membrane oxygenation (ECMO) may be a life-saving procedure. METHODS: An observational study approved by the local ethics committee was carried out. Data from all patients treated with ECMO at the ECMO Center Karolinska for influenza A/H1N1 2009-related severe respiratory failure were analyzed. The main outcome measure was survival three months after discharge from our department. RESULTS: Between July 2009 and January 2010, 13 patients with H1N1 2009 respiratory failure were treated with ECMO. Twelve patients were cannulated for veno-venous ECMO at the referring hospital and transported to Stockholm. One patient was cannulated in our hospital for veno-arterial support. The median ratio of the arterial partial oxygen pressure to the fraction of inspired oxygen (P/F ratio: PaO2 /FiO2) before cannulation was 52.5 (interquartile range 38-60). Four patients were converted from veno-venous to veno-arterial ECMO because of right heart failure (three) or life-threatening cardiac arrhythmias (one). The median maximum oxygen consumption via ECMO was 251 ml/min (187-281 ml/min). Twelve patients were still alive three months after discharge; one patient died four days after discharge due to intracranial hemorrhage. CONCLUSION: Patients treated with veno-venous or veno-arterial ECMO for H1N1 2009-related respiratory failure may have a favorable outcome. Contributing factors may include the possibility of transport on ECMO, conversion from veno-venous (v-v) or veno-arterial (v-a) ECMO if necessary, high-flow ECMO to meet oxygen requirements and active surgery when needed.
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| 14. |
- Skari, H, et al.
(author)
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Congenital diaphragmatic hernia: a survey of practice in Scandinavia
- 2004
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In: Pediatric Surgery International. - : Springer Science and Business Media LLC. - 1437-9813 .- 0179-0358. ; 20:5, s. 309-313
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Journal article (peer-reviewed)abstract
- There is no consensus on the treatment of congenital diaphragmatic hernia (CDH), and practice seems to vary between centres. The main purpose of the present study was to survey current practice in Scandinavia. Thirteen paediatric surgical centres serving a population of about 22 million were invited, and all participated. One questionnaire was completed at each centre. The questionnaire evaluated management following prenatal diagnosis, intensive care strategies, operative treatment, and long-term follow-up. Survival data (1995-1998) were available from 12 of 13 centres. Following prenatal diagnosis of CDH, vaginal delivery and maternal steroids were used at eight and six centres, respectively. All centres used high-frequency oscillation ventilation (HFOV), nitric oxide (NO), and surfactant comparatively often. Five centres had extracorporeal membrane oxygenation (ECMO) facilities, and four centres transferred ECMO candidates. The majority of centres (7/9) always tried HFOV before ECMO was instituted. Surgery was performed when the neonate was clinically stable (11/13) and when no signs of pulmonary hypertension were detected by echo-Doppler (6/13). The repair was performed by laparotomy at all centres and most commonly with nonabsorbable sutures (8/13). Thoracic drain was used routinely at seven centres. Long-term follow-up at a paediatric surgical centre was uncommon (3/13). Only three centres treated more than five CDH patients per year. Comparing survival in centres treating more than five with those treating five or fewer CDH patients per year, there was a tendency towards better survival in the higher-volume centres (72.4%) than in the centres with lower volume (58.7%), p =0.065.
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| 15. |
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| 16. |
- von Bahr, V, et al.
(author)
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Long-term pulmonary function and quality of life in adults after extracorporeal membrane oxygenation for respiratory failure
- 2019
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In: Perfusion. - : SAGE Publications. - 1477-111X .- 0267-6591. ; 34:1_suppl, s. 49-57
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Journal article (peer-reviewed)abstract
- 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.
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| 17. |
- von Bahr, V, et al.
(author)
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The authors reply
- 2018
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In: Critical care medicine. - 1530-0293. ; 46:10, s. E1014-E1015
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Journal article (peer-reviewed)
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| 22. |
- Broome, Michael, et al.
(author)
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Prolonged extracorporeal membrane oxygenation and circulatory support as bridge to lung transplant
- 2008
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In: Annals of Thoracic Surgery. - : Elsevier BV. - 0003-4975 .- 1552-6259. ; 86:4, s. 1357-1360
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Journal article (peer-reviewed)abstract
- A 38-year-old man with progressive alveolitis secondary to polymyositis was treated for 52 days with venovenous and venoarterial extracorporeal membrane oxygenation as a bridge to bilateral lung transplantation. The patient survived, despite multiple complications, and is now back home with good pulmonary function. He is working part-time nearly 3 years post-transplant. This case shows that long-term extracorporeal lung assist is a viable but demanding alternative for bridging patients to pulmonary transplantation. This case also shows that right ventricular failure necessating conversion to veno-arterial assist does not necessarily predict right ventricular failure post-transplant.
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| 28. |
- Burgos, CM, et al.
(author)
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Differences in Outcomes in Prenatally Diagnosed Congenital Diaphragmatic Hernia Compared to Postnatal Detection: A Single-Center Experience
- 2016
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In: Fetal diagnosis and therapy. - : S. Karger AG. - 1421-9964 .- 1015-3837. ; 39:4, s. 241-247
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Journal article (peer-reviewed)abstract
- <b><i>Objectives: </i></b>To compare outcomes in pregnancies with a prenatal detection of congenital diaphragmatic hernia (CDH) with children diagnosed after birth, treated at the same institution, and to determine the ability to predict prognosis through measurements of the observed to expected lung-to-head ratio (O/E LHR). <b><i>Methods:</i></b> This is a retrospective review of all children with CDH treated at our institution during 2006-2014. We compared outcomes of infants referred for surgery after postnatal diagnosis with outcomes of infants with prenatally diagnosed CDH. <b><i>Results:</i></b> In the prenatal group, O/E LHR was significantly different between survivors and deceased patients, with a cutoff at 35% O/E LHR. Survival to discharge and 1-year survival were significantly higher in the postnatal group that required intubation within 24 h; i.e., 92 and 89% versus 85 and 73% in the prenatal group (p < 0.05). There was less need for extracorporeal membrane oxygenation (ECMO), 41 versus 60%, and patch, 41 versus 75% (p < 0.001), in the postnatal group with early diagnosis compared with the prenatal group, respectively. <b><i>Conclusion: </i></b>Children with prenatally diagnosed CDH represent a population with a more severe condition compared to infants diagnosed after birth. They have poorer outcomes with higher needs for ECMO or use of patch, and lower survival rates were observed at an O/E LHR below 35%.
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| 29. |
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| 30. |
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| 31. |
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| 32. |
- Burgos, CM, et al.
(author)
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Maternal Risk Factors and Perinatal Characteristics in Congenital Diaphragmatic Hernia: A Nationwide Population-Based Study
- 2019
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In: Fetal diagnosis and therapy. - : S. Karger AG. - 1421-9964 .- 1015-3837. ; 46:6, s. 385-391
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Journal article (peer-reviewed)abstract
- <b><i>Background:</i></b> The etiology of congenital diaphragmatic hernia (CDH) remains poorly understood. We hypothesize that environmental factors play an important role in the development of CDH. <b><i>Aim:</i></b> The objective of this study was to investigate associated maternal risk factors in pregnancies with CDH. <b><i>Material and Methods:</i></b> 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. <b><i>Results:</i></b> 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, <i>p</i> = 0.01). No association was found between preeclampsia and CDH. <b><i>Conclusions:</i></b> 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.
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| 40. |
- Burstrom, A., et al.
(author)
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Ready for Transfer to Adult Care? A Triadic Evaluation of Transition Readiness in Adolescents With Congenital Heart Disease and Their Parents
- 2019
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In: Journal of Family Nursing. - : SAGE Publications. - 1074-8407 .- 1552-549X. ; 25:3, s. 447-468
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Journal article (peer-reviewed)abstract
- 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.
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| 48. |
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| 49. |
- Falk, L, et al.
(author)
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Differential hypoxemia during venoarterial extracorporeal membrane oxygenation
- 2019
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In: Perfusion. - : SAGE Publications. - 1477-111X .- 0267-6591. ; 34:1_suppl, s. 22-29
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Journal article (peer-reviewed)abstract
- 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.
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