P2.034: Does Pulmonary Hypertension Increase Mortality in Newborns with Omphalocele? A Systematic Review and Meta-analysis

Background: Omphalocele is a congenital midline abdominal wall defect characterized by herniation of the abdominal viscera into a membrane-covered sac, occurring in 1 in 3,000–6,000 live births. Newborns with omphalocele are at risk of respiratory insufficiency, which may lead to pulmonary hypertension (PH). The association between PH in patients with omphalocele and clinical outcomes has been previously studied. We performed a systematic review and meta-analysis evaluating whether pulmonary hypertension increases morbidity and mortality in newborns with omphalocele.

Methods: We conducted a systematic review and meta-analysis evaluating the impact of PH in patients with omphaloceles during the neonatal and infancy periods. We performed literature search in MEDLINE (Pubmed), EMBASE and Cochrane CENTRAL. Patients were divided in two groups depending on the presence of PH. The primary outcomes were mortality and duration of mechanical ventilation. The protocol was prospectively registered in PROSPERO (CRD420251025073).

Results: Out of 323 studies available for screening, four retrospective cohort studies met the inclusion criteria, and 227 patients were included. Among them, 91 (40%) patients had PH and 136 (60%) patients did not have PH. There were no differences between groups regarding birth weight, sex, and gestational age at birth (p > 0.05). Patients with PH had an OR of 10.83 (95% CI 3.57 to 32.86, p < 0.001, I2 = 0) for mortality when compared to patients with no PH. Patients with PH required 21.7 more days of mechanical ventilation (MV) than patients with no PH (95% CI 13.42-29.97, p < 0.001, I2 = 0).

Conclusion: Newborns and infants with omphalocele and PH have higher odds of mortality, and longer duration of MV compared to those without PH. Despite the relatively high prevalence of omphalocele, the association to PH and the implication with outcomes has not been in the spotlight. Further research is needed to better characterize risk factors and optimize management strategies for this vulnerable population to improve overall survivability and long-term outcomes.