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The Management of Pulmonary Hypertension in Infants and Children
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The Management of Pulmonary Hypertension in Infants and Children

A special issue of Children (ISSN 2227-9067). This special issue belongs to the section "Pediatric Pulmonary and Sleep Medicine".

Deadline for manuscript submissions: 20 December 2024 | Viewed by 5375

Special Issue Editors

Dr. Lukas Schroeder

E-Mail Website
Guest Editor
Department of Neonatology and Pediatric Intensive Care, Children’s Hospital, University of Bonn, 53127 Bonn, Germany
Interests: pulmonary hypertension; cardiac dysfunction; echocardiographic assessment; biomarker; congenital diaphragmatic hernia; bronchopulmonary dysplasia
Dr. Florian Kipfmueller

E-Mail Website
Guest Editor
1. Department of Neonatology and Pediatric Intensive Care, Children’s Hospital, University of Bonn, 53127 Bonn, Germany
2. Center for Rare Diseases Bonn, Division of Congenital Malformations, University of Bonn, 53127 Bonn, Germany
Interests: pulmonary hypertension; cardiac dysfunction; echocardiographic assessment; biomarker; congenital diaphragmatic hernia; bronchopulmonary dysplasia

Special Issue Information

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

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Children is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2400 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

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

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Published Papers (3 papers)

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Research

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11 pages, 909 KiB  
Article
The Pulmonary Artery Pulsatility Index Provides No Additional Prognostic Information in Pediatric Pulmonary Arterial Hypertension
by Faye E. Smits, Chantal Lokhorst, Marlies G. Haarman, Mark-Jan Ploegstra, Rolf M. F. Berger and Johannes M. Douwes
Children 2024, 11(10), 1152; https://doi.org/10.3390/children11101152 - 24 Sep 2024
Viewed by 756
Abstract
Background/Objectives: The pulmonary artery pulsatility index (PAPi, calculated as (SPAP − DPAP)/mRAP) has been suggested as a measure of right ventricular–vascular coupling (RVVC) and as a prognostic parameter in cardiovascular conditions, particularly right ventricular failure. This retrospective study investigated the relationship between the [...] Read more.
Background/Objectives: The pulmonary artery pulsatility index (PAPi, calculated as (SPAP − DPAP)/mRAP) has been suggested as a measure of right ventricular–vascular coupling (RVVC) and as a prognostic parameter in cardiovascular conditions, particularly right ventricular failure. This retrospective study investigated the relationship between the PAPi and its components with disease severity parameters, the RVVC, and clinical outcomes in children with pulmonary arterial hypertension (PAH). Methods: We analyzed data from 111 children from the Dutch National Registry with PAH. The PAPi (median 6.0 [3.9–8.3]) was calculated from heart catheterization data and the RVVC was determined as the TAPSE/sPAP ratio via echocardiography (0.25 ± 0.12 mm/mmHg). Disease severity was characterized by clinical, hemodynamic, and laboratory data. Cox proportional hazard modeling assessed the PAPi’s predictive value for transplant-free survival. Results: There was no correlation between the RVVC and PAPi (R = −0.208, p = 0.111, n = 60). The PAPi correlated negatively with uric acid (R = −0.387, p < 0.001) but not with other disease severity parameters. Mean right atrial pressure correlated with multiple disease severity indicators. Transplant-free survival rates at 1, 3, and 5 years were 87%, 79%, and 73%, respectively. Neither the PAPi nor its components correlated with transplant-free survival. Conclusions: In conclusion, the PAPi does not correlate with the RVVC and this study could not demonstrate any prognostic value of the PAPi regarding disease severity or outcomes in children with PAH, challenging its utility in this population. Full article
(This article belongs to the Special Issue The Management of Pulmonary Hypertension in Infants and Children)
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13 pages, 1028 KiB  
Article
Vasoactive Management of Pulmonary Hypertension and Ventricular Dysfunction in Neonates Following Complicated Monochorionic Twin Pregnancies: A Single-Center Experience
by Lukas Schroeder, Leon Soltesz, Judith Leyens, Brigitte Strizek, Christoph Berg, Andreas Mueller and Florian Kipfmueller
Children 2024, 11(5), 548; https://doi.org/10.3390/children11050548 - 3 May 2024
Viewed by 1461
Abstract
Objectives: Twins resulting 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 [...] Read more.
Objectives: Twins resulting 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 complicated 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: The overall PH incidence at day of life (DOL) 1 was 17% and decreased to 6% at DOL 7 (p = 0.013), with no PH findings at DOL 28. The overall incidence of CD was 56% at DOL 1 and decreased strongly until DOL 7 (10%, p = 0.015), with no diagnosis of CD at DOL 28. The use of dobutamine, norepinephrine, and vasopressin at DOL 1 until DOL 7 did not differ between the subgroups, whereas the dosing of milrinone was significantly higher in Group B at DOL 1 (p = 0.043). 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) increased from DOL 1 (12.4 [3/27]) to DOL 2 (14.6 [1/68], p = 0.777), with a significant decrease thereafter as measured at DOL 7 (9.5 [2/30], p = 0.011). Conclusion: Early PH and CD are frequent diagnoses in neonates following a complicated MC twin pregnancy, and an individualized vasoactive treatment strategy is required in the management of these infants. Full article
(This article belongs to the Special Issue The Management of Pulmonary Hypertension in Infants and Children)
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Review

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18 pages, 9434 KiB  
Review
Meconium Aspiration Syndrome, Hypoxic-Ischemic Encephalopathy and Therapeutic Hypothermia—A Recipe for Severe Pulmonary Hypertension?
by Deepika Sankaran, Jessa Rose A. Li and Satyan Lakshminrusimha
Children 2024, 11(6), 673; https://doi.org/10.3390/children11060673 - 1 Jun 2024
Viewed by 2443
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 [...] Read more.
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 the risk of persistent pulmonary hypertension of the newborn (PPHN). Moreover, therapeutic hypothermia (TH), which is the current standard of care for the management of HIE, may increase 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. Full article
(This article belongs to the Special Issue The Management of Pulmonary Hypertension in Infants and Children)
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