Congenital Diaphragmatic Hernia: 10-Year Evaluation of Survival, Extracorporeal Membrane Oxygenation, and Foetoscopic Endotracheal Occlusion in Four High-Volume Centres

Abstract

Background: Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly with significant mortality. Objectives: The aim of this study was to determine if there were trends in survival over the last decade and to compare patient populations, treatment options, and survival rates between 4 high-volume centres, and hence determine which factors were associated with survival. Methods: In 4 high-volume CDH centres from the CDH EURO Consortium, data from all CDH patients born between 2004 and 2013 were analysed. The predictive value of variables known at birth and the influence of centre-specific treatments (extracorporeal membrane oxygenation, ECMO, and foetoscopic endotracheal occlusion, FETO) on survival were evaluated in multivariable logistic regression analyses. Results: Nine hundred and seventy-five patients were included in the analysis, of whom 274 (28.1%) died. ECMO was performed in 259 patients, of whom 81 (31.3%) died. One hundred and forty-five patients (14.9%) underwent FETO, and from those 76 patients (52.4%) survived. Survival differed significantly between years (p = 0.006) and between the 4 centres (p < 0.001). In the multivariable logistic regression analysis, lung-to-head ratio, gestational age at birth, ECMO, centre of birth, and year of birth were significantly associated with survival, whereas FETO was not. Conclusions: The patient populations were different between centres, which influenced outcomes. There was a significant variability in survival over time and between centres, which should be taken into consideration in the planning of future trials.

Congenital diaphragmatic hernia: Impact of contemporary management strategies on perinatal outcomes

“…Although ECMO has been widely used as “rescue therapy” in infants with CDH in other centers, in particular those with right‐sided CDH, it has only been used in two infants in the setting of CDH in our state in the past 20 years. The overall survival rate of live births since the changes in postnatal management of CDH was introduced in 2003 was 75.8%, which is comparable with the 71.9% survival rate in large European centers where 14.9% of fetuses underwent FETO, and 31.3% of neonates had ECMO . Nonetheless, it is plausible that there exist confounding factors, such as low o/e LHR or defect laterality, which may have negatively influenced prenatal counselling and consequent pregnancy management and in part contributed to the comparable survival rates between WA and European centers, in the absence of FETO and the infrequent use of ECMO.…”

“…In recent years, the shift from early surgical intervention to intensive preoperative supportive care, such as gentle mechanical ventilation with permissive hypercapnia, pharmacological use of inhaled nitric oxide (iNO) and intravenous sildenafil, and extracorporeal membrane oxygenation (ECMO), has resulted in an increase in the postnatal survival rates for CDH, typically 72%-90%. [8][9][10] Nonetheless, the incidence of long-term morbidities, including respiratory and gastrointestinal complications, remains significant. 11 There have been several population-based epidemiological studies on the outcomes and prognosis of CDH, 12,13 including one from our unit in Western Australia evaluating outcomes from 1991 to 2002.…”

Congenital diaphragmatic hernia has a better prognosis when associated with a hernia sac

“…Surgical repair is achieved after hemodynamic stabilization. Survival of neonates with CDH has improved in the last 2 decades from 67.7 to 71.4%, although it still varies from 1 center to another …”

Prediction of ECMO and Mortality in Neonates with Congenital Diaphragmatic Hernia Using the SNAP-II Score

“…Despite advances in the management of CDH newborns, mortality remains high, even in specialized high-volume centers [22]. Early risk stratification is crucial in CDH newborns to identify patients at need for ECMO therapy which is only available in selected centers [16].…”

“…Congenital diaphragmatic hernia (CDH) occurs approximately in 1/4000 live birth [13]. Despite advances in the management of these patients, mortality ranges between 20 and 40 % even in specialized high-volume centers [22]. Pulmonary hypertension (PH) and lung hypoplasia are major contributors to morbidity and mortality in CDH newborns [5].…”