High Prevalence of Pulmonary Hypertension Complicates the Care of Infants with Omphalocele

Abstract

Background: Omphalocele is one of the most common abdominal wall defects. Many newborn infants born with omphalocele present with significant respiratory distress at birth, requiring mechanical ventilatory support, and have clinical evidence of pulmonary hypertension. Little information exists on the prevalence of and risk factors associated with pulmonary hypertension in this cohort of infants. Objectives: To describe the prevalence of and risk factors associated with pulmonary hypertension among infants with omphalocele. Methods: This is a multicenter retrospective chart review of demographic data and clinical characteristics of infants with omphalocele admitted to the neonatal intensive care units of Loma Linda University Children's Hospital and Children's Mercy Hospital between 1994 and 2011. Echocardiogram images were reviewed for pulmonary hypertension, and statistical analyses were performed to identify risk factors associated with the presence of pulmonary hypertension. Results: Pulmonary hypertension was diagnosed in 32/56 (57%) infants with omphalocele. Compared to infants without pulmonary hypertension, infants with pulmonary hypertension were more likely to have a liver-containing defect (16/32 [50%] vs. 5/24 [21%], p = 0.03), require intubation at birth (18/32 [56%] vs. 6/24 [17%], p = 0.03), and die during initial hospitalization (12/32 [38%] vs. 2/24 [8%], p = 0.01). Conclusion: The majority of infants with omphalocele have evidence of pulmonary hypertension which is associated with increased mortality. Echocardiograms to screen for pulmonary hypertension should be obtained at ≥2 days of life in infants with omphalocele, especially in those with liver within the omphalocele sac and/or in those infants who require intubation at birth to screen for pulmonary hypertension.

Comprehensive Echocardiographic Assessment of Ventricular Function and Pulmonary Pressure in the Neonatal Omphalocele Population

“…Furthermore, on echocardiography, patients with GO had increased sPAP/ sBP ratio compared with their non-GO counterpart. Many previous studies have described increased pulmonary pressures in the immediate postnatal life of newborns with GO, [1][2][3] supporting the findings in our cohort of patients. GO status has been associated with increased requirements for medical and surgical care, respiratory failure, prolonged hospitalization, and mortality.…”

“…Initial based on aforementioned cut-offs for TAPSE, RV-pGLS, and RV-FAC on initial echocardiography were significantly associated with an increased risk of death for the overall patient population, as exemplified on the Kaplan-Meier survival curves (►Figs. [1][2][3]. The logistic regression analysis results with death as outcome and TAPSE as exposure (adjusted for gestational age and GO status) are presented in ►Table 4.…”

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Pulmonary hypertension (PH) has been reported as a commonly associated finding in neonates with omphalocele and has been associated with increased mortality. [1][2][3][4] This is particularly of a concern for patients with a giant omphalocele (GO), as there is a concern for underlying pulmonary hypopla-sia in the context of mechanical restriction during fetal growth. 1,2 Some have advocated for echocardiography in the early postnatal period to assess PH in newborns with this condition.

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Mortality in neonates with giant omphalocele subjected to a surgical technique in Barranquilla, Colombia from 1994 to 2019

“…For Baerg et al, pulmonary hypertension is a predictor of mortality when this condition occurs between the second and seventh days of life 23 . The above findings demonstrate that pulmonary hypertension is a significant and underestimated complication and highlight the importance of its early (after the second day of life) and periodic monitoring by echocardiography among patients with giant omphalocele 14,24 .…”

Rapid development of pulmonary hypertension during treatment of paediatric cancer

“…The secondary diagnosis of pulmonary hypertension has also been noted in association with other disorders as well. 4,5 Despite recognition of the linkage between cancer and pulmonary hypertension in adults and reporting of the pulmonary side effects from cancer and treatment in children, little information exists on the association of pulmonary hypertension and cancer in children. 6,7 Here we report on a cohort of children whose cancer treatment was complicated by pulmonary hypertension, emphasising the effectiveness of pulmonary hypertension therapy and recoverability.…”