Pulmonary hypertension in giant omphalocele infants

Background: Pulmonary hypoplasia has been described in cases of giant omphalocele (GO), although pulmonary hypertension (PH) has not been extensively studied in this disorder. In the present study, we describe rates and severity of PH in GO survivors who underwent standardized prenatal and postnatal care at our institution.

Methods: A retrospective chart review was performed for all patients in our pulmonary hypoplasia program with a diagnosis of GO. Statistical significance was calculated using Fisher's exact test and Mann-Whitney test (p<0.05).

Results: Fifty-four patients with GO were studied, with PH diagnosed in twenty (37%). No significant differences in gender, gestational ages, birth weight, or Apgar scores were associated with PH. Patients diagnosed with PH were managed with interventions, including high frequency oscillatory ventilation, and nitric oxide. Nine patients required long-term pulmonary vasodilator therapy. PH was associated with increased length of hospital stay (p<0.001), duration of mechanical ventilation (p=0.008), and requirement for tracheostomy (p=0.0032). Overall survival was high (94%), with significantly increased mortality in GO patients with PH (p=0.0460). Prenatal imaging demonstrating herniation of the stomach into the defect was significantly associated with PH (p=0.0322), with a positive predictive value of 52%.

Conclusions: In this series, PH was observed in 37% of GO patients. PH represents a significant complication of GO, and management of pulmonary dysfunction is a critical consideration in improving clinical outcomes in these patients.