There is emerging evidence supporting ventricular function as a prognostic factor in congenital diaphragmatic hernia (CDH). The present systematic review and meta-analysis aimed to determine the predictive value of early ventricular function for survival and extracorporeal membrane oxygenation (ECMO) requirement in newborns with CDH. PubMed, Google Scholar, Cochrane Central Register, Clinical Trial Registry, and Opengrey were accessed. Studies evaluating associations between echocardiographic ventricular function measured ≤ 48 h after birth and survival or ECMO requirement were included. Two independent authors extracted the following data: study and participant characteristics, prognostic factors, and outcome-related data. Eleven studies met the inclusion criteria. Five studies reported on survival, two on ECMO, and four on both outcomes. A moderate risk of bias was found in most of the studies, mainly because of selection, prognostic factors, and confounding biases. For survival (899 participants), pooled sensitivity and specificity were 86% (95% confidence interval [CI], 77-92%) and 44% (95% CI, 25-65%), respectively, in normal left ventricular function. For ECMO need (815 participants), pooled sensitivity and specificity were 39.8% (95% CI, 27-52%) and 88% (95% CI, 80-96%), respectively, in left ventricular dysfunction. Overall certainty of the evidence was graded very low for survival and low for ECMO. Inconsistent reporting of echocardiographic measurements and lack of adjustment for confounding factors were major limitations.Conclusion: Early ventricular dysfunction is a potential prognostic factor in CDH. Standardized echocardiographic measurement reporting and high-quality studies are needed to further elucidate its prognostic significance. What is Known: • Evidence supports the predictive value of echocardiographic measurements in CDH ≤ 24-48 h post-birth. • Ventricular dysfunction has been proposed as a prognostic risk factor. What is New: • Right and left ventricular functions were promising predictors of survival and ECMO requirement in neonates with CDH. • Test characteristics of ventricular function were determined as predictors of survival or need for ECMO. Specific echocardiographic markers of ventricular function can be valuable in determining prognosis.

Infants with congenital diaphragmatic hernia (CDH) usually have pulmonary hypoplasia and persistent pulmonary hypertension of the newborn (PPHN) leading to hypoxemic respiratory failure (HRF). Pulmonary hypertension associated with CDH is frequently resistant to conventional pulmonary vasodilator therapy including inhaled nitric oxide (iNO). Increased pulmonary vascular resistance (PVR) can lead to right ventricular overload and dysfunction. In patients with CDH, left ventricular dysfunction, either caused by right ventricular overload or a relative underdevelopment of the left ventricle, is associated with poor prognosis. Milrinone is an intravenous inotrope and lusitrope (enhances cardiac systolic contraction and diastolic relaxation respectively) with pulmonary vasodilator properties and has been shown anecdotally to improve oxygenation in PPHN. Milrinone is commonly used during the management of CDH although no randomized trials have been performed to test its efficacy. Thirty percent of infants with CDH in the Children\'s Hospital Neonatal Database (CHND) and 22% of late-preterm and term infants with CDH in the Pediatrix database received milrinone. In the recently published VICI trial, 84% of patients with CDH received a vasoactive medication. In the current pilot trial, neonates with an antenatal or postnatal diagnosis of CDH will be randomized to receive milrinone or placebo to establish safety of this medication in CDH and test its efficacy in improving oxygenation.

BACKGROUND:

Congenital diaphragmatic hernia, although rare (1 per 2-4,000 births), is associated with high mortality and cost. Opinion regarding the timing of surgical repair has gradually shifted from emergent repair to a policy of stabilization using a variety of ventilatory strategies prior to operation. Whether delayed surgery is beneficial remains controversial.

OBJECTIVES:

To summarize the available data regarding whether surgical repair in the first 24 hours after birth rather than later than 24 hours of age improves survival to hospital discharge in infants with congenital diaphragmatic hernia who are symptomatic at or immediately after birth.

SEARCH STRATEGY:

Search of Medline (1966-1999), Embase (1978-1999) and the Cochrane databases using the terms "congenital diaphragmatic hernia" and "surg*"; citations search, and contact with experts in the field to locate other published and unpublished studies.

SELECTION CRITERIA:

Studies were eligible for inclusion if they were randomized or quasi-randomized trials that addressed infants with CDH who were symptomatic at or shortly after birth, comparing early (<24 hours) vs late (>24 hours) surgical intervention, and evaluated mortality as the primary outcome.

DATA COLLECTION AND ANALYSIS:

Data were collected regarding study methods and outcomes including mortality, need for ECMO and duration of ventilation, both from the study reports and from personal communication with investigators. Analysis was performed in accordance with the standards of the Cochrane Neonatal Review Group.

MAIN RESULTS:

Two trials met the pre-specified inclusion criteria for this review. Both were small trials (total n<90) and neither showed any significant difference between groups in mortality. Meta-analysis was not performed because of significant clinical heterogeneity between the trials.

REVIEWER'S CONCLUSIONS:

There is no clear support for either immediate (within 24 hours of birth) or delayed (until stabilized) repair of congenital diaphragmatic hernia, but a substantial advantage to either one cannot be ruled out. A large, multicenter randomized trial would be needed to answer this question.

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Background: Congenital diaphragmatic hernia, although rare (1 per 2-4,000 births), is associated with high mortality and cost. Opinion regarding the timing of surgical repair has gradually shifted from emergent repair to a policy of stabilization using a variety of ventilatory strategies prior to operation. Whether delayed surgery is beneficial remains controversial. Objectives: To summarize the available data regarding whether surgical repair in the first 24 hours after birth rather than later than 24 hours of age improves survival to hospital discharge in infants with congenital diaphragmatic hernia who are symptomatic at or immediately after birth. Search methods: Search of MEDLINE (1966 to Sept 2003), EMBASE (1978 to Oct 2003) and the Cochrane Central Register of Controlled Trials (CENTRAL, The Cochrane Library, Issue 3, 2003); citations search, and contact with experts in the field to locate other published and unpublished studies. This search was updated in 2009. Selection criteria: Studies were eligible for inclusion if they were randomized or quasi-randomized trials that addressed infants with CDH who were symptomatic at or shortly after birth, comparing early (< 24 hours) vs late (> 24 hours) surgical intervention, and evaluated mortality as the primary outcome. Data collection and analysis: Data were collected regarding study methods and outcomes including mortality, need for ECMO and duration of ventilation, both from the study reports and from personal communication with investigators. Analysis was performed in accordance with the standards of the Cochrane Neonatal Review Group. Main results: Two trials met the pre-specified inclusion criteria for this review. Both were small trials (total n<90) and neither showed any significant difference between groups in mortality. Meta-analysis was not performed because of significant clinical heterogeneity between the trials. Authors' conclusions: There is no clear evidence which favors delayed (when stabilized) as compared with immediate (within 24 hours of birth) timing of surgical repair of congenital diaphragmatic hernia, but a substantial advantage to either one cannot be ruled out. A large, multicenter randomized trial would be needed to answer this question.

Objective: To compare outcomes of open and endosurgical neonatal congenital diaphragmatic hernia (CDH) repairs. Background: Historically a surgical emergency, neonatal CDH repair is now deferred pending stabilization of characteristically labile cardiopulmonary physiology. Usually accomplished via laparotomy, surgical repair may acutely worsen lung function; conversely, by reducing the visceral hernia, surgery might improve it. Theoretically, endosurgical repair could minimize deleterious effects of surgery while garnering benefits from decompressing the CDH lung. As endosurgical repair gains popularity, it is important to investigate whether or not minimally-invasive neonatal CDH repair has benefits. Methods: We searched Medline, Embase, and Cochrane Trials databases for studies comparing open with endosurgical CDH repair. Non-neonatal series and reports without comparison groups were excluded. References from papers and conference proceedings were also hand searched. Meta-analysis used a fixed effects model and was reported in accordance with PRISMA. Results: We included 3 studies (1 unpublished; none randomized); all compared thoracoscopic and open CDH repair and together described 143 patients. All studies had limitations, including use of historical controls. Demographics, CDH sidedness, APGAR and associated anomaly prevalence were similar between groups. For endosurgical repair, recurrence was higher (RR: 3.2 [1.1, 9.3], P = 0.03) and operative time longer (WMD 50 minutes [32, 69], P < 0.00001). Survival and patch usage were not different between open and endosurgical groups. Conclusions: Neonatal thoracoscopic CDH repair has greater recurrence rates and operative times but similar survival and patch usage compared with open surgery. A prospective registry for all such cases would guide development of trials (Stage 2b; IDEAL recommendations). Copyright © 2010 by Lippincott Williams & Wilkins.

The health-related quality of life (HRQoL) of survivors of congenital diaphragmatic hernia (CDH) is presently unknown. Literature prior to 2015 suggests that CDH survivors have worse HRQoL compared with the general population. However, due to changes in the diagnosis, management, and follow-up of CDH patients, this may no longer be true. The objective of this review was to determine what recent literature suggests regarding the HRQoL of CDH survivors. We reviewed all studies published between April 9, 2015, and April 6, 2020, that described the HRQoL of CDH survivors. Studies were identified using PubMed, and the references of the identified papers were searched for additional studies eligible for inclusion. Seven studies describing HRQoL in CDH survivors using validated measures of HRQoL for children, PedsQL (Pediatric Quality of Life Inventory), KIDSCREEN-52, and Comprehensive Quality of Life Scale - School Version were examined. The findings were disparate. One study suggested that CDH survivors had better than average HRQoL scores, three studies found patients and controls had equivalent scores, and two studies reported poor outcomes for CDH patients. The final study found no effect of disease severity on HRQoL, as determined by prenatal ultrasound. Evidence published between 2015 and 2020 suggests that CDH patients have an HRQoL equivalent to the general population.

BACKGROUND:

Repair of congenital diaphragmatic hernia (CDH) has changed from an emergent procedure to a delayed procedure in the last decade. Many other aspects of management have also evolved since the first successful repair. However, most reports are from single institutions. The lack of a large multicenter database has hampered progress in the management of congenital diaphragmatic hernia (CDH) and makes determination of the current standard difficult.

METHODS:

The CDH study group was formed in 1995 to collect data from multiple institutions in North America, Europe, and Australia. Participating centers completed a registry form on all live-born infants with CDH during 1995 and 1996. Demographic information, data about surgical management, and outcome were collected for all patients.

RESULTS:

Sixty-two centers participated, with 461 patients entered. Overall survival was 280 of 442 patients (63%) where survival was recorded. The defect was left-sided in 78%, right-sided in 21%, and bilateral in 1%. A subcostal approach was used in 91% of patients, with pleural drainage used in 76%. A patch of some kind was used in just over half (51%) of the patients, with polytetrafluoroethylene being the most commonly used material (81%) in those patients with a patch. The mean surgical time was 102 minutes, with an average blood loss of 14 mL (range, 0 to 500 mL). The overwhelming majority of patients underwent repair between 6:00 AM and 6:00 PM (289 of 329, 88%). Nineteen percent of patients had surgical repair on extracorporeal membrane oxygenation (ECMO) at a mean time of 170 hours into the ECMO course (range, 10 to 593 hours). The mean age at surgery in patients not treated with ECMO was 73 hours (range, 1 to 445 hours).

CONCLUSIONS:

The multicenter nature of this report makes it a snapshot of current management. The data would indicate that prosthetic patching of the defect has become common, that after-hours repair is infrequent, and that delayed surgical repair has become the preferred approach in many centers. Furthermore, the mean survival rate of 63% indicates that despite decades of individual effort, the CDH problem is far from solved. This highlights the need for a centralized database and cooperative multicenter studies in the future.

BACKGROUND:

Congenital diaphragmatic hernia (CDH) is one of the high-risk diseases in pediatric surgery, especially in neonates with symptom presentation within 6 hours after birth. Opinion regarding the time of surgery has gradually shifted from immediate repair to a policy of stabilization and delayed repair. Whether delayed surgery is beneficial remains controversial.

OBJECTIVE:

To evaluate the outcomes regarding whether delayed surgical repair improves survival in CDH neonates, who are symptomatic immediately after delivery, is more beneficial than immediate surgery.

MATERIAL AND METHOD:

Data were obtained by searching MEDLINE (1966-2002) and the Cochrane Database, Issue 2, 2003 using the term "congenital diaphragmatic hernia" and "surgery". Inclusion criteria were randomized controlled trial (RCT), prospective trial (PT), retrospective analysis (RA) and meta-analysis (MA). Information from the literature was analyzed by the computer program of Epi Info Version 3. Statistical significance was reliable at the level of p < 0.05.

RESULTS:

Twenty-five studies were obtained and RCT/RA was 2/23. Analysis of results of CDH management revealed that pre-operative stabilization and delayed surgery improved the survival rate in 14 of 25 in the literature (p < 0.05), while the remaining 11 articles showed no statistical difference of survival between immediate and delayed surgery (p > 0.05).

CONCLUSION:

From the evidence-based analysis, the results of CDH management between immediate versus delayed surgery were unclear. From the reviewer's experience at the Queen Sirikit National Institute of Child Health, the strategy of pre-operative stabilization and delayed surgery had better improved survival of CDH than immediate surgery.

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