Primary study

Unclassified

Does the ex utero intrapartum treatment to extracorporeal membrane oxygenation procedure change morbidity outcomes for high-risk congenital diaphragmatic hernia survivors?

Year 2017
Journal Journal of pediatric surgery
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PURPOSE:

In high-risk congenital diaphragmatic hernia (CDH), significant barotrauma or death can occur before extracorporeal membrane oxygenation (ECMO) can be initiated. We previously examined ex utero intrapartum treatment (EXIT)-to-ECMO in our most severe CDH patients, but demonstrated no survival advantage. We now report morbidity outcomes in survivors of this high-risk cohort to determine whether EXIT-to-ECMO conferred any benefit.

METHODS:

All CDH survivors with <15% predicted lung volume (PPLV) from September 1999 to December 2010 were included. We recorded prenatal imaging, defect size, and pulmonary, nutritional, cardiac, and neurodevelopmental outcomes.

RESULTS:

Seventeen survivors (8 EXIT-to-ECMO, 9 non-EXIT) had an average PPLV of 11.7%. Eight of 9 non-EXIT received ECMO within 2days. There were no significant defect size differences between groups, mostly left-sided (13/17) and type D (12/17). Average follow-up was 6.7years (0-13years). There were no statistically significant differences in outcomes, including supplemental oxygen, diuretics, gastrostomy, weight-for-age Z scores, fundoplication, pulmonary hypertension, stroke or intracranial hemorrhage rate, CDH recurrence, and reoperation. No survivor in our cohort was neurologically devastated. All had mild motor and/or speech delay, which improved in most.

CONCLUSIONS:

In this pilot series of severe CDH survivors, EXIT-to-ECMO confers neither significant survival nor long-term morbidity benefit.

LEVEL OF EVIDENCE:

Level III treatment study.

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Primary study

Unclassified

Analysis of perceived risk and satisfaction with telematic follow-up in patients and families of congenital diaphragmatic hernia patients during SARS-CoV-2 pandemia.

Year 2023
Journal Journal of healthcare quality research
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BACKGROUND:

The impact of COVID-19 in families and patients with congenital diaphragmatic hernia (CDH) is unknown, this situation has generated uncertainty not only in family members but also in the optimal outpatient follow-up. Telehealth has become a fundamental tool for the follow-up during the pandemic. The objective of this survey is to evaluated the impact of SARS-CoV-2 in families and patients with CDH and the satisfaction with telematic follow-up.

METHODS:

Telephone survey of patient's caregivers with CHD, aged 1-16 years, followed in neonatal surgery outpatients, from January 31, 2020 to November 15, 2020. The ethical clearance for this study was taken from the Clinical Research Ethics Committee of our Research Institute vide letter number VHIR/239283/01.01.2021.

RESULTS:

81 surveys of 100 patients with active follow-up were carried out. There were no refusals in any contacted parents. There were 30 contacts (37%), 44.8% at school and 27.6% from cohabiting family members. Four infections (4.9%) were diagnosed, half symptomatic. In 40 patients (49.4%) the follow-up was telematic, with a mean score of 3.1±1.3 out of 5. For future controls, 65% prefer presential follow-up, 25% alternate and 10% telematics. 50.6% reported greater anxiety and 34.6% (28/81) extreme measures of isolation, being more accentuated in the group of 3-6 years (p<0.05).

CONCLUSION:

The impact of COVID19 in patients with CHD is not greater than in the general pediatric population. Although the incorporation of the telehealth was well valued, most of the caregivers prefer the face-to-face outpatient follow-up.

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Primary study

Unclassified

The VICI-trial: high frequency oscillation versus conventional mechanical ventilation in newborns with congenital diaphragmatic hernia: an international multicentre randomized controlled trial.

Year 2011
Journal BMC pediatrics
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<b>

BACKGROUND:

</b>Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly of the diaphragm resulting in pulmonary hypoplasia and pulmonary hypertension. It is associated with a high risk of mortality and pulmonary morbidity. Previous retrospective studies have reported high frequency oscillatory ventilation (HFO) to reduce pulmonary morbidity in infants with CDH, while others indicated HFO to be associated with worse outcome. We therefore aimed to develop a randomized controlled trial to compare initial ventilatory treatment with high-frequency oscillation and conventional ventilation in infants with CDH.<b>Methods/design: </b>This trial is designed as a multicentre trial in which 400 infants (200 in each arm) will be included. Primary outcome measures are BPD, described as oxygen dependency by day 28 according to the definition of Jobe and Bancalari, and/or mortality by day 28. All liveborn infants with CDH born at a gestational age of over 34 weeks and no other severe congenital anomalies are eligible for inclusion. Parental informed consent is asked antenatally and the allocated ventilation mode starts within two hours after birth. Laboratory samples of blood, urine and tracheal aspirate are taken at the first day of life, day 3, day 7, day 14 and day 28 to evaluate laboratory markers for ventilator-induced lung injury and pulmonary hypertension.<b>

DISCUSSION:

</b>To date, randomized clinical trials are lacking in the field of CDH. The VICI-trial, as the first randomized clinical trial in the field of CDH, may provide further insight in ventilation strategies in CDH patient. This may hopefully prevent mortality and morbidity.<b>Trial Registration: </b>Netherlands Trial Register (NTR): NTR1310.

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Primary study

Unclassified

An international multicenter randomized controlled trial of high frequency oscillation versus conventional mechanical ventilation in infants with congenital diaphragmatic hernia: the VICI-trial

Year 2008
Registry of Trials Overview of Medical Research in the Netherlands
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Primary study

Unclassified

The effect of maternal betamethasone and fetal tracheal occlusion on pulmonary vascular morphometry in fetal rabbits with surgically induced diaphragmatic hernia: A placebo controlled morphologic study

Year 2009
Journal Prenatal Diagnosis
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Objectives: We studied the vascular effects of betamethasone (BM) and/or tracheal occlusion (TO) in fetal rabbits with surgically induced congenital diaphragmatic hernia (CDH). Methods: At day 23 (pseudoglandular phase; term = 31 d), 54 ovarian-end fetuses from 27 does underwent induction of CDH. Thirteen did receive either 0.05 mg/kg BM, on days 28 and 29 with a 24-h interval, or 14 saline [controls (CTR)]. At day 28, one ovarian-end fetus underwent TO and harvesting was at term. In total, we compared (ANOVA) lung-to-body weight ratio (LBWR) and vascular morphometric indices in survivors from the following groups (n - number alive at delivery): CDH (9); CDH + TO (10); unoperated controls (14); CDH + BM (10); CDH + TO (9); controls CTR + BM (13). Results: Maternal BM had no effect on LBWR. LBWR was comparable to normal in CDH fetuses undergoing TO. Both TO and BM have an effect on medial thickening due to CDH which is larger when both interventions are combined. Conclusions: Both TO and BM lessen peripheric muscularization present in CDH lungs and their effect is cumulative. Copyright © 2009 John Wiley & Sons, Ltd.

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Structured summary of systematic reviews

Unclassified

What is the best prosthetic material for patch repair of congenital diaphragmatic hernia? Comparison and meta-analysis of porcine small intestinal submucosa and polytetrafluoroethylene (Provisional abstract)

Year 2012
Authors Romao RL , Nasr A , Chiu PP , Langer JC
Journal Journal of Pediatric Surgery
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Primary study

Unclassified

Physiological-based cord clamping versus immediate cord clamping for infants born with a congenital diaphragmatic hernia (PinC): study protocol for a multicentre, randomised controlled trial.

Year 2022
Journal BMJ open
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INTRODUCTION:

Pulmonary hypertension is a major determinant of postnatal survival in infants with a congenital diaphragmatic hernia (CDH). The current care during the perinatal stabilisation period in these infants might contribute to the development of pulmonary hypertension after birth-in particular umbilical cord clamping before lung aeration. An ovine model of diaphragmatic hernia demonstrated that cord clamping after lung aeration, called physiological-based cord clamping (PBCC), avoided the initial high pressures in the lung vasculature while maintaining adequate blood flow, thereby avoiding vascular remodelling and aggravation of pulmonary hypertension. We aim to investigate if the implementation of PBCC in the perinatal stabilisation period of infants born with a CDH could reduce the incidence of pulmonary hypertension in the first 24 hours after birth.

METHODS AND ANALYSIS:

We will perform a multicentre, randomised controlled trial in infants with an isolated left-sided CDH, born at ≥35.0 weeks. Before birth, infants will be randomised to either PBCC or immediate cord clamping, stratified by treatment centre and severity of pulmonary hypoplasia on antenatal ultrasound. PBCC will be performed using a purpose-built resuscitation trolley. Cord clamping will be performed when the infant is considered respiratory stable, defined as a heart rate >100 bpm, preductal oxygen saturation >85%, while using a fraction of inspired oxygen of <0.5. The primary outcome is pulmonary hypertension diagnosed in the first 24 hours after birth, based on clinical and echocardiographic parameters. Secondary outcomes include neonatal as well as maternal outcomes.

ETHICS AND DISSEMINATION:

Central ethical approval was obtained from the Medical Ethical Committee of the Erasmus MC, Rotterdam, The Netherlands (METC 2019-0414). Local ethical approval will be obtained by submitting the protocol to the regulatory bodies and local institutional review boards.

TRIAL REGISTRATION NUMBER:

NCT04373902.

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Primary study

Unclassified

Antenatal sildenafil administration to prevent pulmonary hypertension in congenital diaphragmatic hernia (SToP-PH): study protocol for a phase I/IIb placenta transfer and safety study.

Year 2018
Journal Trials
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BACKGROUND:

Congenital diaphragmatic hernia is an orphan disease with high neonatal mortality and significant morbidity. An important cause for this is pulmonary hypertension, for which no effective postnatal therapy is available to date. An innovative strategy aiming at treating or preventing pulmonary hypertension more effectively is urgently needed. Prenatal sildenafil administration to expectant mothers prevented fetal and neonatal vascular changes leading to pulmonary hypertension in several animal models, and is, therefore, a promising approach. Before transferring this antenatal medical approach to the clinic, more information is needed on transplacental transfer and safety of sildenafil in humans.

METHODS:

This is a randomized, investigator-blinded, double-armed, parallel-group, phase I/IIb study with as a primary objective to measure the in-vivo transplacental transfer of sildenafil in women in the second and early third trimester of pregnancy (sub-study 1; weeks: 20.0-32.6) and at term (sub-study 2; weeks: 36.6-40). Participants will be randomized to two different sildenafil doses: 25 or 75 mg. In sub-study 1, a single dose of the investigational product will be administered to women undergoing termination of pregnancy, and maternal and fetal blood samples will be collected for determination of sildenafil concentrations. In sub-study 2, sildenafil will be administered three times daily from 3 days before planned delivery until actual delivery, following which maternal and umbilical cord samples will be collected. Proxies of maternal and fetal tolerance as well as markers of fetal pulmonary vasodilation will also be measured.

DISCUSSION:

This is the first study evaluating in-vivo transplacental passage of sildenafil in humans.

TRIAL REGISTRATION:

EU Clinical Trials Register 2016-002619-17, validated on 12 August 2016. Trial sponsor: UZ Leuven, Herestraat 49, 3000 Leuven.

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Primary study

Unclassified

Impacts of Respiratory Muscle Training on Respiratory Functions, Maximal Exercise Capacity, Functional Performance, and Quality of Life in School-Aged Children with Postoperative Congenital Diaphragmatic Hernia.

Year 2020
Journal Disease markers
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OBJECTIVES:

Congenital diaphragmatic hernia (CDH) is a birth defect affecting the respiratory functions, functional performance, and quality of life (QOL) in school-aged children. Rarely have studies been conducted to evaluate the impacts of respiratory muscle training on school-aged children with postoperative CDH. The current study was designed to evaluate the impacts of respiratory muscle training on respiratory function, maximal exercise capacity, functional performance, and QOL in these children.

METHODS:

This study is a randomized control study. 40 children with CDH (age: 9-11 years) were assigned randomly into two groups. The first group conducted an incentive spirometer exercise combined with inspiratory muscle training (study group, n = 20), whereas the second group conducted only incentive spirometer exercise (control group, n = 20), thrice weekly for twelve consecutive weeks. Respiratory functions, maximal exercise capacity, functional performance, and pediatric quality of life inventory (PedsQL) were assessed before and after the treatment program. Results. Regarding the posttreatment analysis, the study group showed significant improvements in all outcome measures (FVC%, p < 0.001; FEV1%, p = 0.002; VO2max, p = 0.008; VE/VCO2 slope, p = 0.002; 6-MWT, p < 0.001; and PedsQL, p < 0.001), whereas the control group did not show significant changes (p > 0.05).

CONCLUSION:

Respiratory muscle training may improve respiratory functions, maximal exercise capacities, functional performance, and QOL in children with postoperative CDH. Clinical commendations have to be considered to include respiratory muscle training in pulmonary rehabilitation programs in children with a history of CDH.

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Primary study

Unclassified

Multicentre, randomised controlled trial of physiological-based cord clamping versus immediate cord clamping in infants with a congenital diaphragmatic hernia (PinC): statistical analysis plan.

Year 2024
Journal Trials
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BACKGROUND:

Infants born with congenital diaphragmatic hernia (CDH) are at high risk of respiratory insufficiency and pulmonary hypertension. Routine practice includes immediate clamping of the umbilical cord and endotracheal intubation. Experimental animal studies suggest that clamping the umbilical cord guided by physiological changes and after the lungs have been aerated, named physiological-based cord clamping (PBCC), could enhance the fetal-to-neonatal transition in CDH. We describe the statistical analysis plan for the clinical trial evaluating the effects of PBCC versus immediate cord clamping on pulmonary hypertension in infants with CDH (PinC trial).

, DESIGN:

The PinC trial is a multicentre, randomised controlled trial in infants with isolated left-sided CDH, born >= 35.0 weeks of gestation. The primary outcome is the incidence of pulmonary hypertension in the first 24 h after birth. Maternal outcomes include estimated maternal blood loss. Neonatal secondary outcomes include mortality before discharge, extracorporeal membrane oxygenation therapy, and number of days of mechanical ventilation. Infants are 1:1 randomised to either PBCC or immediate cord clamping using variable random permutated block sizes (4-8), stratified by treatment centre and estimated severity of pulmonary hypoplasia (i.e. mild/moderate/severe). At least 140 infants are needed to detect a relative reduction in pulmonary hypertension by one third, with 80% power and 0.05 significance level. A chi-square test will be used to evaluate the hypothesis that PBCC decreases the occurrence of pulmonary hypertension. This plan is written and submitted without knowledge of the collected data. The trial has been ethically approved.

, TRIAL REGISTRATION:

ClinicalTrials.gov NCT04373902 (registered April 2020). Copyright © 2024. The Author(s).

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