Pulmonary arterial hypertension in neonates and children post open-heart surgery: assessment and management including extracorporeal membrane oxygenation (ECMO) — a narrative review

Background

Congenital heart disease (CHD) that requires heart surgery is common. Severe postoperative pulmonary hypertension (PH) occurs in 2% of cardiac procedures and is associated with high morbidity and mortality. Pulmonary arterial hypertension (PAH) is defined by a mean pulmonary artery pressure (mPAP) exceeding 20 mmHg at rest along with normal pulmonary arterial wedge pressure (PAWP) of ≤ 15 mmHg and pulmonary vascular resistance (PVR) > 2 Wood units (WUs). When assessed and anticipated before surgery, it is managed proactively through standard perioperative measures or pharmacological interventions.

Main body

We review the literature on PAH in PubMed from January 2014 to December 2024 with a focus on PAH in the perioperative context of CHD and its management within the pediatric cardiac intensive care unit (PCICU), including PAH management during venoarterial extracorporeal membrane oxygenation (VA-ECMO). Definitions and diagnostic criteria for PAH are well established, and numerous expert statements and management guidelines are documented in the literature; however, there is a limited focus on critical care settings compared with outpatient settings. Additionally, there are no clear guidelines for initiating pulmonary vasodilators after cardiac surgery or on VA-ECMO. To address this gap, we propose a pathway to provide management guidance for PAH in neonates and children following cardiac surgery in intensive care unit (ICU) settings and on VA-ECMO.

Conclusion

Implementing a management pathway for PAH for critical care patients admitted to the PCICU may help reduce morbidity and mortality as well as waiting times on ECMO, thereby improving patient outcomes.