A clinical prediction rule for the severity of congenital diaphragmatic hernias in newborns

Congenital diaphragmatic hernia (CDH) is a condition with a highly variable outcome. Some infants have a relatively mild disease process, whereas others have significant pulmonary hypoplasia and hypertension. Identifying high-risk infants postnatally may allow for targeted therapy. Data were obtaine...

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Bibliographic Details
Published in:Pediatrics (Evanston) 2014-08, Vol.134 (2), p.e413-e419
Main Authors: Brindle, Mary Elizabeth, Cook, Earl Francis, Tibboel, Dick, Lally, Pamela A, Lally, Kevin P
Format: Article
Language:English
Subjects:
Analysis
Apgar Score
Babies
Birth weight
Comorbidity
Complications and side effects
Decision Support Techniques
Diaphragm
Diaphragmatic hernia
Female
Health aspects
Heart Defects, Congenital - epidemiology
Hernia
Hernia, Diaphragmatic - epidemiology
Hernia, Diaphragmatic - mortality
Hernia, Diaphragmatic - therapy
Hernias
Hernias, Diaphragmatic, Congenital
Humans
Hypertension
Hypertension, Pulmonary - diagnostic imaging
Hypertension, Pulmonary - epidemiology
Infant, Newborn
Infants
Male
Outcome and process assessment (Health Care)
Outcome and process assessment (Medical care)
Pediatrics
Pulmonary hypertension
Risk Assessment
Risk factors
Severity of Illness Index
Ultrasonography
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Summary:Congenital diaphragmatic hernia (CDH) is a condition with a highly variable outcome. Some infants have a relatively mild disease process, whereas others have significant pulmonary hypoplasia and hypertension. Identifying high-risk infants postnatally may allow for targeted therapy. Data were obtained on 2202 infants from the Congenital Diaphragmatic Hernia Study Group database from January 2007 to October 2011. Using binary baseline predictors generated from birth weight, 5-minute Apgar score, congenital heart anomalies, and chromosome anomalies, as well as echocardiographic evidence of pulmonary hypertension, a clinical prediction rule was developed on a randomly selected subset of the data by using a backward selection algorithm. An integer-based clinical prediction rule was created. The performance of the model was validated by using the remaining data in terms of calibration and discrimination. The final model included the following predictors: very low birth weight, absent or low 5-minute Apgar score, presence of chromosomal or major cardiac anomaly, and suprasystemic pulmonary hypertension. This model discriminated between a population at high risk of death (∼50%) intermediate risk (∼20%), or low risk (
ISSN:0031-4005
1098-4275
DOI:10.1542/peds.2013-3367