Pulmonary Hypertension in Infants with Omphalocele

BACKGROUND: Many infants born with an omphalocele present with significant respiratory distress at birth requiring mechanical ventilatory support. Some infants present with clinical evidence of pulmonary hypertension which may complicate their overall outcome. To date, no data exist on the prevalence of pulmonary hypertension in this cohort of infants.

OBJECTIVE: To describe the prevalence of pulmonary hypertension among infants with an omphalocele and identify risk factors associated with the presence of pulmonary hypertension.

DESIGN/METHODS: A total of 33/46 (72%) infants with omphalocele admitted to the LLUCH NICU between 1994-2011 had echocardiograms available for review. Demographic data and clinical characteristics were collected by retrospective chart review. Echocardiogram(s) were reviewed by a single pediatric cardiologist for pulmonary hypertension based on presence of flattening of the interventricular septum and/or tricuspid regurgitant jet with estimated RV pressure >40mmHg. Data were summarized and compared for the pulmonary hypertension versus the no pulmonary hypertension cohort using Fisher's exact test, two-sample t-test, or Mann-Whitney test as appropriate. Significance was set at p<0.05.

RESULTS: Pulmonary hypertension was diagnosed in 23/33 (70%) infants with an omphalocele. Most infants with pulmonary hypertension were female 14/23 (61%) with mean gestational age 35.7 +/- 3.4 weeks. Presence of liver in the omphalocele sac was more prevalent in the pulmonary hypertension cohort compared to the no pulmonary hypertension cohort, 18/23 (78%) vs 3/10 (30%), respectively, p= 0.02.