The Management of Pulmonary Hypertension in Infants and Children

Dear Colleagues,

Pulmonary hypertension (PH) is one of the major contributors to short- and long-term morbidity and mortality in critically ill infants and children. In particular, infants and children with underlying congenital or acquired cardiovascular and pulmonary diseases are at risk when PH is diagnosed, with the need for long-term pharmaceutical treatment.

Therefore, the diagnosis and treatment of PH play an important role in its management in infants and children. Different diagnostic approaches, including biomarker profiles, echocardiographic assessments, and strategies such as multi-omic profiling, are becoming increasingly available and popular for the diagnosis of PH. Furthermore, multiple pharmaceutical treatment strategies have been evaluated in recent years in infants and children, offering insights into treatment effects and drug profiles.

This Special Issue will provide innovative and informative data on recent advantages in PH management, including new aspects of diagnosis and treatment of PH in pediatric populations. This Special Issue will focus on offering insights from retrospective and prospective clinical research of modern PH management to improve infant’s and children’s future medical care.

Dr. Lukas Schroeder
Dr. Florian Kipfmueller
Guest Editors

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• pulmonary hypertension
• biomarker profiles
• echocardiographic assessment
• multi-omic profiling
• pharmaceutical treatment
• infants
• children

Title: right ventricle remodeling in Pulmonary Hypertension
Authors: Zengkong Dai
Affiliation: Kaohsiung Medical University, Kaohsiung, Taiwan

Title: Vasoactive management of pulmonary hypertension and ventricular dysfunction in neonates following complicated monochorionic twin pregnancies: a single-center experience
Authors: Lukas Schroeder; Leon Soltesz; Judith Leyens; Brigitte Strizek; Christoph Berg; Andreas Mueller; Florian Kipfmueller
Affiliation: Department of Neonatology and Pediatric Intensive Care Medicine, University Children´s Hospital Bonn, Germany
Abstract: Objectives - Twins suffering from a complicated monochorionic (MC) twin pregnancy are at risk for postnatal evolution of pulmonary hypertension (PH) and cardiac dysfunction (CD). Both pathologies are important contributors to short- and long-term morbidity in these infants. The aim of the present retrospective single-center cohort study was to evaluate the need for vasoactive treatment for PH and CD in these neonates. Methodology - In-born neonates following a complicates MC twin pregnancy admitted to the department of neonatology of the University Children´s Hospital Bonn (UKB) between October 2019 and December 2023 were screened for study inclusion. Finally, 70 neonates were included in the final analysis, with 37 neonates subclassified as recipient twins (Group A) and 33 neonates as donor twins (Group B). Results - Overall PH incidence at day of life (DOL) 1 was 17% and decreased to 6% at DOL 7, with no PH findings at DOL 28. The overall incidence of CD was 56% at DOL 1 and decreased strongly until DOL 7 (10%), with no diagnosis of CD at DOL 28. Use of dobutamine, norepinephrine, and vasopressin at DOL 1 until DOL 7 did not differ between subgroups, whereas the dosing of milrinone was significantly higher in Group B at DOL 1. Inhaled nitric oxide (iNO) was used in 16% of the cohort and a levosimendan therapy was administered in 34% of the neonates. One third of the cohort was treated with oral beta-blockers and in 10% an intravenous beta-blockade (landiolol) was administered. The maximum Levosimendan-Vasoactive-Inotropic Score (LVISmax) at DOL 1 was 12.4 (3/27), at DOL 2 14.6 (1/68), and at DOL 9.5 (2/30). Conclusion – Early PH and CD are frequent diagnosis in neonates following a complicated MC twin pregnancy and an individualized vasoactive treatment strategy is required in the management of these infants.

Title: Meconium aspiration syndrome, Hypoxic Ischemic Encephalopathy and Therapeutic Hypothermia - A Recipe for Severe Pulmonary Hypertension?
Authors: Deepika Sankaran; Jessa Li; Satyan Lakshminrusimha
Affiliation: Division of Neonatology, University of California, Davis, Sacramento, California, USA 95817
Abstract: Hypoxic ischemic encephalopathy (HIE) is the leading cause of mortality among term newborns globally. Infants born through meconium-stained amniotic fluid are at risk of developing meconium aspiration syndrome (MAS) and HIE. Simultaneous occurrence of MAS and HIE is a perilous combination for newborns due to risk of persistent pulmonary hypertension of the newborn (PPHN). Moreover, therapeutic hypothermia (TH) which is the current standard of care for management of HIE may increase the pulmonary vascular resistance (PVR) and worsen PPHN. Infants with MAS and HIE require close cardiorespiratory and hemodynamic monitoring for PPHN. Therapeutic strategies including oxygen supplementation, ventilation, use of surfactant, inhaled nitric oxide and other pulmonary vasodilators, and systemic vasopressors play a critical role in the management of PPHN in MAS, HIE and TH. While TH reduces death or disability in infants with HIE, infants with MAS and HIE undergoing TH need close hemodynamic monitoring for PPHN.