Congenital diaphragmatic hernia: endothelin-1, pulmonary hypertension, and disease severity.

ABSTRACT:

Rationale

Endothelin-1 (ET1) is dysregulated in pulmonary hypertension (PH). It may be important in the pathobiology of congenital diaphragmatic hernia (CDH).

Objectives

We hypothesized that ET1 levels in the first month would be higher in infants with CDH who subsequently expired or were discharged on oxygen (poor outcome). We further hypothesized that ET1 levels would be associated with concurrent severity of PH.

Methods

We sampled plasma at 24 to 48 hours, and 1, 2, and 4 weeks of age in 40 prospectively enrolled newborns with CDH. We performed echocardiograms to estimate pulmonary artery pressure at less than 48 hours of age and weekly to 4 weeks. PH was classified in relationship to systemic blood pressure (SBP): less than 2/3 SBP, 2/3 SBP-systemic is related to

SUBMITTER: Keller RL 

PROVIDER: S-EPMC2937245 | biostudies-literature | 2010 Aug

REPOSITORIES: biostudies-literature