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Management of pulmonary hypertension in infants with congenital diaphragmatic hernia
1Section of Neonatology, Department of Pediatrics, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, CO, USA.
Insights
Congenital diaphragmatic hernia (CDH) infants face severe pulmonary hypertension (PH) due to lung and heart issues. Pulmonary vasodilator treatments like inhaled nitric oxide (iNO) have evolving roles in managing PH, impacting survival and long-term outcomes.
Area of Science:
- Pediatric Surgery
- Neonatology
- Cardiology
Background:
- Congenital diaphragmatic hernia (CDH) involves abdominal contents herniating into the chest, compressing developing lungs and heart.
- Severe cases present with lung hypoplasia, pulmonary hypertension (PH), and left ventricular (LV) dysfunction, leading to high mortality.
- Managing PH in CDH is complex due to evolving cardiopulmonary pathophysiology in neonates.
Purpose of the Study:
- To review the evolving management strategies for pulmonary hypertension (PH) in infants with congenital diaphragmatic hernia (CDH).
- To discuss the role and limitations of pulmonary vasodilator therapies, including inhaled nitric oxide (iNO), in different phases of PH management in CDH.
- To highlight the challenges in optimizing treatment for PH and its long-term impact in CDH survivors.
Main Methods:
- Literature review of studies on congenital diaphragmatic hernia (CDH) and pulmonary hypertension (PH) management.
- Analysis of the physiological changes and treatment responses in neonates with CDH.
- Examination of the efficacy and timing of pulmonary vasodilator use in acute and subacute phases.
Main Results:
- Pulmonary vasodilators, such as inhaled nitric oxide (iNO), have limited early use due to LV dysfunction but are crucial for subacute PH management in CDH.
- iNO can stabilize patients for extracorporeal membrane oxygenation (ECMO) and treat persistent PH, potentially improving survival.
- Chronic PH and vascular abnormalities can persist into childhood, influencing long-term outcomes in CDH patients.
Conclusions:
- The management of PH in CDH requires a dynamic approach, adapting to the infant's evolving cardiopulmonary status.
- Pulmonary vasodilator therapy timing is critical, with subacute iNO showing significant benefits for persistent PH.
- Further research is needed to understand the long-term effects of therapies like iNO, sildenafil, and bosentan on chronic PH in CDH survivors.
Abstract:
In infants with congenital diaphragmatic hernia (CDH), a posterolateral diaphragmatic defect results in herniation of abdominal contents into the chest and compression of the intrathoracic structures. In the most severe cases, hypoplasia of the ipsilateral and contralateral lungs, severe pulmonary hypertension (PH) and left ventricular (LV) hypoplasia/dysfunction all contribute to increased mortality. The management of PH in CDH is complicated by structural and functional changes in the heart, pulmonary vasculature, airways and lung parenchyma; consequently, determining optimal management strategies is challenging. Treatment of PH in patients with CDH changes as the underlying pathophysiology evolves in the days and weeks after birth. During the early transition, the use of pulmonary vasodilators is limited by LV structural and functional abnormalities, and pulmonary vasodilators such as inhaled nitric oxide (iNO) may have a limited role (for example, stabilization for extracorporeal membrane oxygenation (ECMO), treatment of marked preductal desaturation and treatment of PH as LV performance improves). In contrast, subacute treatment of PH in CDH with iNO has an important role in recurrent or persistent PH and potentially improves survival. Chronic PH and vascular abnormalities may persist into childhood in patients with CDH, contributing to late mortality. It is unclear how pulmonary vasodilator therapies, such as iNO, sildenafil and bosentan, will modulate late outcomes in CDH with late/chronic PH.
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