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Giant omphalocele associated pulmonary hypertension: A retrospective study
Tai-Xiang Liu1, Li-Zhong Du1, Xiao-Lu Ma1
1Department of NICU, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, National Children's Regional Medical Center, Children's Hospital, Hangzhou, China.
Insights
Pulmonary hypertension (PH) complicates giant omphalocele (GO) in 35.8% of neonates, increasing mortality. Early PH screening via echocardiography is crucial for high-risk infants with GO requiring staged surgical repair.
Area of Science:
- Neonatal surgery
- Pediatric cardiology
- Congenital anomalies
Background:
- Giant omphalocele (GO) is a significant congenital abdominal wall defect.
- Pulmonary hypertension (PH) is a known complication of GO, associated with increased mortality.
- Management of GO, especially with PH, poses challenges for neonatologists and pediatric surgeons.
Purpose of the Study:
- To review and summarize clinical features and prognosis of neonates with GO complicated by PH.
- To identify risk factors associated with PH in neonates with GO.
- To understand the mortality predictors in neonates with GO and PH.
Main Methods:
- Retrospective analysis of medical records of infants with GO (July 2015 - June 2020).
- Patients divided into PH and non-PH groups, and further into death and survival groups.
- Binary logistic regression used to analyze risk factors for PH.
Main Results:
- 24 out of 67 neonates (35.8%) with GO had PH.
- PH was associated with intubation, pulmonary dysplasia, PDA, staged operations, longer ventilation, oxygen, PN, and NICU/hospital stay.
- A staged operation was an independent risk factor for PH; low birth weight, high pulmonary arterial pressure, and refractory PH increased mortality.
Conclusions:
- PH is a serious complication significantly increasing mortality and morbidity in infants with GO.
- Routine echocardiographic screening for PH is recommended for neonates with GO, particularly those requiring staged surgical repair.
- Infants with low weight, severe, or refractory PH face a higher risk of death.
Background:
Omphalocele is a common congenital defect of the abdominal wall, management of giant omphalocele (GO) is particularly for pediatric surgeons and neonatologists worldwide. The current study aimed to review and summarize the clinical features and prognosis in neonates with GO complicated with pulmonary hypertension (PH), which is associated with increased mortality, while in hospital.
Materials And Methods:
Medical records of infants with GO between July 2015 and June 2020 were retrospectively analyzed. The patients enrolled were divided into PH and non-PH groups based on the presence or absence of PH, and patients with PH were divided into death and survival groups based on survival status. Clinical characteristics and outcomes were compared between groups, respectively. The risk factors for PH were analyzed by binary logistic regression.
Results:
In total, 67 neonates were identified as having GO and 24 (35.8%) were complicated with PH. Infants with PH were associated with intubation within 24 h after birth (p = 0.038), pulmonary dysplasia (p = 0.020), presence of patent ductus arteriosus (PDA; p = 0.028), a staged operation (p = 0.002), longer mechanical ventilation days (p < 0.001), oxygen requirement days (p < 0.001), parenteral nutrition (PN) days (p < 0.001), length of neonatal intensive care unit (NICU) or hospital stay (p = 0.001 and 0.002, respectively), and mortality (p = 0.001). The results of multivariable logistic regression analysis revealed that a staged operation was independently associated with PH. In addition, PH patients with lower birth weight, higher peak of pulmonary arterial systolic pressure, and refractory to pulmonary vasodilators (PVD) had increased mortality.
Conclusion:
Pulmonary hypertension is a serious complication and significantly increases the mortality and morbidities in infants with a GO. In addition, early and serial assessment of PH by echocardiography should be a routine screening scheme, especially in the neonatal omphalocele population who required a staged surgical repair. Clinicians should be aware that infants with PH who had low weight, severe and refractory PH have a higher risk of death.
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