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Published on: October 19, 2013
Pulmonary hypertension in giant omphalocele infants
Emily A Partridge1, Brian D Hanna2, Howard B Panitch3
1Department of Surgery, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, 19104, United States.
Insights
Pulmonary hypertension (PH) affects 37% of giant omphalocele (GO) survivors, increasing mortality and hospital stay. Early detection and management of PH are crucial for improving outcomes in GO patients.
Area of Science:
- Neonatalogy
- Pediatric Surgery
- Pulmonology
Background:
- Pulmonary hypoplasia is a known complication of giant omphalocele (GO).
- Pulmonary hypertension (PH) in GO has not been extensively studied.
- This study investigates PH rates and severity in GO survivors.
Purpose of the Study:
- To determine the incidence and severity of PH in giant omphalocele survivors.
- To identify factors associated with PH in GO.
- To evaluate the impact of PH on clinical outcomes in GO patients.
Main Methods:
- Retrospective chart review of GO patients in a pulmonary hypoplasia program.
- Statistical analysis using Fisher's exact test and Mann-Whitney test.
- Standardized prenatal and postnatal care protocols were followed.
Main Results:
- PH was diagnosed in 37% of 54 GO patients.
- PH was associated with longer hospital stays, mechanical ventilation, and tracheostomy.
- Prenatal imaging of stomach herniation predicted PH with 52% accuracy.
- Mortality was significantly higher in GO patients with PH (p=0.0460).
Conclusions:
- Pulmonary hypertension is a significant complication in 37% of giant omphalocele cases.
- PH management is critical for improving outcomes in GO patients.
- Prenatal imaging may aid in early PH identification.
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.
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