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Liver herniation in congenital diaphragmatic hernia is associated with delayed resolution of pulmonary hypertension
Uthaya Kumaran Kanagaraj1, Mimi T Y Kuan1, Michael Castaldo1
1Department of Pediatrics, University of British Columbia, Vancouver, BC, Canada.
Insights
Infants with congenital diaphragmatic hernia and liver herniation experience delayed pulmonary hypertension resolution. These infants also require longer hospital stays and more invasive ventilation.
Area of Science:
- Neonatal Medicine
- Pediatric Cardiology
- Surgical Neonatology
Background:
- Congenital diaphragmatic hernia (CDH) is associated with severe pulmonary hypertension (PH), impacting mortality and morbidity.
- Intrathoracic liver herniation (liver-up) in CDH predicts survival and the need for extracorporeal membrane oxygenation.
- Limited data exists on the timeline of PH resolution in CDH infants, particularly concerning liver herniation.
Purpose of the Study:
- To determine the time to PH resolution in infants with liver-up versus liver-down CDH.
- To compare resource utilization, including hospitalization duration and ventilation days, between these groups.
Main Methods:
- Retrospective study of CDH infants (January 2018 - March 2021) in a quaternary NICU.
- Targeted neonatal echocardiography (TnEcho) used for PH assessment, defining resolution by PDA shunting or septal shape.
- Serial TnEcho assessments performed regularly, especially around the perioperative period.
Main Results:
- Nineteen CDH infants were analyzed; 9 (47.4%) had liver-up.
- Median time to PH resolution was significantly longer in the liver-up group (20.5 days) compared to the liver-down group (11.5 days).
- Liver-up infants had longer median hospital stays (113 vs. 31 days), invasive ventilation days (19 vs. 7.5 days), and higher rates of tube dependence at discharge (88.9% vs. 20.0%).
Conclusions:
- Congenital diaphragmatic hernia infants with liver herniation experience delayed pulmonary hypertension resolution.
- Liver herniation in CDH is associated with prolonged hospitalization and increased need for invasive ventilation.
- These findings highlight the significant impact of liver herniation on clinical outcomes and resource utilization in CDH.
Background:
Severe and persistent pulmonary hypertension (PH) predicts mortality and short-term pulmonary morbidity in infants with congenital diaphragmatic hernia (CDH). Intrathoracic liver herniation (liver-up) is a predictor of survival and increased need for extracorporeal membrane oxygenation. There is a paucity of data on the timeline of PH resolution in infants with and without liver herniation. We aim to determine the time to PH resolution in infants with liver-up and liver-down CDH, and to describe the resource utilization among these infants.
Methods:
We conducted a retrospective study of CDH infants in a quaternary neonatal intensive care unit between January 2018 and March 2021 who received targeted neonatal echocardiography (TnEcho). Our primary outcome of PH resolution was defined echocardiographically by left-to-right shunting at the patent ductus arteriosus (PDA) or rounded interventricular septum on parasternal short axis view if the PDA was closed. Serial TnEcho assessments were performed every 1-2 days around the perioperative period and then every 2-3 weeks as clinically indicated.
Results:
Nineteen CDH infants were evaluated, of which 9 (47.4 %) infants were in the liver-up group. Baseline characteristics such as birth weight, gestational age, and sex were comparable in both groups. The median time to PH resolution was prolonged among infants with liver herniation (20.5 vs. 11.5 days, p = 0.005). The median length of stay and invasive ventilation days were longer in liver-up group (113 vs. 31 days, p = 0.017) and (19 vs. 7.5 days, p = 0.033), respectively. The proportion of infants demonstrating tube dependence for feeding at discharge was higher in the liver-up group compared with the liver-down group (8 [88.9 %] vs. 2 [20.0 %], p = 0.005).
Conclusion:
CDH infants with liver herniation experience a delayed resolution of pulmonary hypertension. Furthermore, these infants require longer hospitalization and more days on invasive ventilation.
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