Original Article
Outcomes of Congenital Diaphragmatic Hernia in the Modern Era of Management

https://doi.org/10.1016/j.jpeds.2012.12.036Get rights and content

Objective

To identify clinical factors associated with pulmonary hypertension (PH) and mortality in patients with congenital diaphragmatic hernia (CDH).

Study design

A prospective cohort of neonates with a diaphragm defect identified at 1 of 7 collaborating medical centers was studied. Echocardiograms were performed at 1 month and 3 months of age and analyzed at a central core by 2 cardiologists independently. Degree of PH and survival were tested for association with clinical variables using Fischer exact test, χ2, and regression analysis.

Results

Two hundred twenty patients met inclusion criteria. Worse PH measured at 1 month of life was associated with higher mortality. Other factors associated with mortality were need for extracorporeal membrane oxygenation, patients inborn at the treating center, and patients with a prenatal diagnosis of CDH. Interestingly, patients with right sided CDH did not have worse outcomes.

Conclusions

Severity of PH is associated with mortality in CDH. Other factors associated with mortality were birth weight, gestational age at birth, inborn status, and need for extracorporeal membrane oxygenation.

Section snippets

Methods

Subjects were recruited as part of the Diaphragmatic Hernia Research and Exploration, Advancing Molecular Science (DHREAMS) study (http://www.cdhgenetics.com). The DHREAMS study is a prospective cohort of neonates with a diaphragm defect identified at 1 of 7 collaborating medical centers. Columbia University began enrollment in January 2005, and recruitment at 6 other sites (Washington University Medical Center/St. Louis Children's Hospital, University of Pittsburgh, Cincinnati Children's

Results

A total of 220 of the 313 eligible cases born at participating centers during the study time period consented to enrollment in the DHREAMS study (Table I). The average gestational age was 37 weeks. White, non-Hispanic patients comprised 54.5% of the study population; 56.8 % were male; 84.1% of the diaphragmatic lesions were left sided; and 61.8% of the patients had an isolated CDH. Of the patients with other anomalies, congenital heart disease was the most common in 21.4% of the entire cohort.

Discussion

Recent advances in the post-natal care of children with CDH at specialized centers have improved survival and decreased the need for ECMO.3 However, with improved survival, many of the long-term morbidities of CDH have been exposed. The leading cause of morbidity and mortality in patients with CDH is severe hypoxic respiratory failure, resulting from pulmonary hypoplasia and abnormal pulmonary vascular development and vasoreactivity, leading to PH.3, 9, 10 Recent studies have correlated the

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Supported by the National Institute of Health (National Institute of Child Health and Development R-01 HD057036 - Identification of Novel Genes for Congenital Diaphragmatic Hernia) and Columbia University's Clinical and Translational Science Award (UL1 RR024156 from National Center for Advancing Translational Sciences-National Center for Research Resources/National Institutes of Health). The authors declare no conflicts of interest.

Contributed equally.

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