Home » Health » Early prostacyclin use in newborns with pulmonary hypertension associated with congenital diaphragmatic hernia (CDH) was associated with reduced need for extracorporeal life support, according to a registry-based cohort study. However, prostacyclin did not affect in-hospital mortality. CDH, a birth defect that can prevent the lungs from developing fully, affects one in every 3,600 babies. Newborns with CDH who receive ECLS have higher mortality, so strategies to decrease ECLS use are important. Effective medical treatment of pulmonary hypertension associated with CDH presents a challenge due to the way patients respond to vasoactive agents.

Early prostacyclin use in newborns with pulmonary hypertension associated with congenital diaphragmatic hernia (CDH) was associated with reduced need for extracorporeal life support, according to a registry-based cohort study. However, prostacyclin did not affect in-hospital mortality. CDH, a birth defect that can prevent the lungs from developing fully, affects one in every 3,600 babies. Newborns with CDH who receive ECLS have higher mortality, so strategies to decrease ECLS use are important. Effective medical treatment of pulmonary hypertension associated with CDH presents a challenge due to the way patients respond to vasoactive agents.

Diaphragmatic hernia, a rare congenital malformation, can cause life-threatening respiratory distress in newborns. Traditional treatment involves mechanical ventilation and prolonged hospitalization, often with the use of extracorporeal membrane oxygenation (ECMO) or other life support measures. However, a recent study suggests that early administration of intravenous prostacyclin may reduce the need for such interventions, potentially improving outcomes and reducing healthcare costs. This article will explore the findings of the study and discuss the implications for the management of diaphragmatic hernia in neonates.


Prostacyclin administered during the first week of life reduced the need for extracorporeal life support (ECLS) in newborns with pulmonary hypertension associated with congenital diaphragmatic hernia (CDH), according to a recent study published in JAMA Pediatrics. However, the study found no difference in in-hospital mortality. Researchers used data from the CDH Study Group registry to match a control group with a treatment group. In the matched cohort study, babies who received prostacyclin had a lower need for ECLS (23.3% vs 42.9%). They also had a shorter duration of ECLS (8.6 vs 12.6 days). With a reduction in ECLS use, there could be potential benefits in avoiding resource-intensive care among high-risk neonates with CDH.

CDH is a birth defect that can cause breathing difficulties for newborns, as it might prevent the lungs from developing completely. The newborns with CDH who receive ECLS often experience a mortality rate ranging from 2.7 to 4 times higher, after adjusting for disease severity. Effective medical treatment of pulmonary hypertension linked with CDH can be challenging as these patients respond to vasoactive agents differently than those with pulmonary hypertension due to other pathophysiologic processes.

The researchers noted that prostacyclins decrease pressure in the pulmonary arteriolar bed via vasodilation and can have a titrating effect on clinical outcomes. Prior literature surrounding the use of prostacyclin for CDH neonates is primarily limited to small-sample safety and feasibility studies. Though data were collected prospectively, the study was observed retrospectively, and there was no standardisation of management and care across CDHSG institutions.

The study also highlights the need for further investigation as there are currently no widely accepted guidelines for prostacyclin users in patients with CDH. Moreover, no distinction existed between the early treatment and control patients for in-hospital mortality, as propensity score matching may have addressed the differences in disease severity among these groups.

In conclusion, early prostacyclin usage could reduce the need for extracorporeal life support in newborns with pulmonary hypertension associated with CDH. However, there are currently no widely accepted guidelines for prostacyclin usage in patients with CDH. More investigations of the potential benefits and risks in this area could continue to improve outcomes for patients with CDH.


In conclusion, the use of early prostacyclin therapy has proven to be a promising solution to reduce the need for life support in infants with diaphragmatic hernia. This breakthrough treatment has opened up new possibilities for improving the outcomes and overall quality of life for these young patients. With further research and development, we can look forward to even more effective and innovative treatments for this condition. It is clear that early intervention is crucial in ensuring optimal outcomes for infants with diaphragmatic hernia, and we can only hope to continue to see positive results in the years to come.

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