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Medicina
Special Issues
Focused Update on Pulmonary Hypertension in Children
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Focused Update on Pulmonary Hypertension in Children

A special issue of Medicina (ISSN 1648-9144). This special issue belongs to the section "Pediatrics".

Deadline for manuscript submissions: closed (30 September 2020) | Viewed by 12924

Special Issue Editors

Dr. Bibhuti Das

E-Mail Website
Guest Editor
Baylor College of Medicine, Texas Children’s Hospital, Houston, TX, USA
Interests: pulmonary hypertension in children; cardiomyopathy; heart failure; heart transplantation; exercise testing; adult congenital heart disease; pediatric cardiology
Prof. Dunbar Ivy

E-Mail Website
Co-Guest Editor
University of Colorado, Children’s Hospital of Colorado, Denver, CO, USA
Interests: pulmonary hypertension; pulmonary vascular disease; congenital heart disease; altitude medicine; pediatric cardiology

Special Issue Information

Dear Colleagues,

The field of pulmonary hypertension is rapidly progressing. With the most recent changes to the classification and definition of pulmonary hypertension and its subtypes (World Symposium on pulmonary hypertension in Nice 2018) and new knowledge for the optimal diagnosis and treatment of infants, children, and young adults with pulmonary hypertension (including CHD/Eisenmenger and single ventricle physiology/Fontan), since the publication of the 2015 pulmonary hypertension guideline in children by AHA, there has been a need to update and discuss the challenging issues inherent to pediatric pulmonary hypertension.  

Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic diseases in neonates, infants, and older children, and contributes to significant morbidity and mortality. Even though our knowledge and understanding of this pathology has increased in the last few years, many aspects need further clarification. Thus, there is a need for a focussed update on the topic.

This Special Issue of Medicina will act as a forum to increase our understanding of pulmonary hypertension in children, by sharing knowledge about incompletely understood and controversial aspects.

Both original research articles and literature reviews will be considered for publication.

Dr. Bibhuti Das
Guest Editors

Prof. Dunbar Ivy
Co-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. Medicina 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 1800 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

  • pros-and cons of new criteria of pulmonary hypertension in children
  • pulmonary hypertension in Fontan physiology
  • pulmonary hypertension in children with sickle cell disease
  • pulmonary hypertension in left heart disease: the role of the ventricular assist device
  • pulmonary hypertension with Scimitar syndrome
  • podiatric Eissenmenger syndrome due to complex CHD
  • segmental pulmonary hypertension
  • newer diagnostic modalities (ECHO and CMR) and their application to children with pulmonary hypertension
  • pulmonary hypertension with left heart disease and subsequent heart alone vs heart and lung transplantation

Published Papers (4 papers)

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Review

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8 pages, 1765 KiB  
Review
COVID-19 and Pulmonary Hypertension in Children: What Do We Know So Far?
by Bibhuti B Das
Medicina 2020, 56(12), 716; https://doi.org/10.3390/medicina56120716 - 19 Dec 2020
Cited by 11 | Viewed by 4838
Abstract
The interplay between coronavirus disease 2019 (COVID-19) and pulmonary hypertension (PH) in children is unknown. Adults with PH are at potential risk for severe complications and high mortality due to associated comorbidities. It is difficult to extrapolate the outcomes of COVID-19 in adults [...] Read more.
The interplay between coronavirus disease 2019 (COVID-19) and pulmonary hypertension (PH) in children is unknown. Adults with PH are at potential risk for severe complications and high mortality due to associated comorbidities. It is difficult to extrapolate the outcomes of COVID-19 in adults to pediatric PH patients. Overall, a small number of COVID-19 cases is reported in patients with preexisting PH. Several factors may be responsible for the low incidence of COVID-19 in children with PH. Pulmonary hypertension is a rare disease, testing is not universal, and patients may have followed more rigorously the Center for Disease Control’s guidelines recommended for personal protection with mask-wearing, social distancing, and hand sanitization through ongoing health education. The small number of COVID-19 cases in patients with preexisting PH does not support that PH is protective for COVID-19. However, medications used to treat PH may have some protection against COVID-19. This review discusses the pathophysiology of PH occurring with COVID-19, differences between children and adults with COVID-19, strategies for management of preexisting PH in children during the ongoing pandemic, and its impact within the field of PH. Full article
(This article belongs to the Special Issue Focused Update on Pulmonary Hypertension in Children)
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34 pages, 2520 KiB  
Review
Focused Update on Pulmonary Hypertension in Children—Selected Topics of Interest for the Adult Cardiologist
by Sulaima Albinni, Manfred Marx and Irene M. Lang
Medicina 2020, 56(9), 420; https://doi.org/10.3390/medicina56090420 - 19 Aug 2020
Viewed by 3603
Abstract
Pulmonary hypertensive vascular disease (PHVD), and pulmonary hypertension (PH), which is a broader term, are severe conditions associated with high morbidity and mortality at all ages. Treatment guidelines in childhood are widely adopted from adult data and experience, though big differences may exist [...] Read more.
Pulmonary hypertensive vascular disease (PHVD), and pulmonary hypertension (PH), which is a broader term, are severe conditions associated with high morbidity and mortality at all ages. Treatment guidelines in childhood are widely adopted from adult data and experience, though big differences may exist regarding aetiology, concomitant conditions and presentation. Over the past few years, paediatric aspects have been incorporated into the common guidelines, which currently address both children and adults with pulmonary hypertension (PH). There are multiple facets of PH in the context of cardiac conditions in childhood. Apart from Eisenmenger syndrome (ES), the broad spectrum of congenital heart disease (CHD) comprises PH in failing Fontan physiology, as well as segmental PH. In this review we provide current data and novel aspects on the pathophysiological background and individual management concepts of these conditions. Moreover, we focus on paediatric left heart failure with PH and its challenging issues, including end stage treatment options, such as mechanical support and paediatric transplantation. PH in the context of rare congenital disorders, such as Scimitar Syndrome and sickle cell disease is discussed. Based on current data, we provide an overview on multiple underlying mechanisms of PH involved in these conditions, and different management strategies in children and adulthood. In addition, we summarize the paediatric aspects and the pros and cons of the recently updated definitions of PH. This review provides deeper insights into some challenging conditions of paediatric PH in order to improve current knowledge and care for children and young adults. Full article
(This article belongs to the Special Issue Focused Update on Pulmonary Hypertension in Children)
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Other

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7 pages, 2025 KiB  
Case Report
Interventional Cardiovascular Magnetic Resonance Imaging (iCMR) in an Adolescent with Pulmonary Hypertension
by Surendranath R. Veeram Reddy, Yousef Arar, Tarique Hussain, Gerald Greil, Luis Zabala and Bibhuti B. Das
Medicina 2020, 56(12), 636; https://doi.org/10.3390/medicina56120636 - 24 Nov 2020
Cited by 2 | Viewed by 1635
Abstract
The interventional cardiac magnetic resonance imaging (iCMR) catheterization procedure is feasible and safe for children and adults with pulmonary hypertension and congenital heart defects (CHD). With iCMR, the calculation of pulmonary vascular resistance (PVR) in children with complex CHD with multilevel shunt lesions [...] Read more.
The interventional cardiac magnetic resonance imaging (iCMR) catheterization procedure is feasible and safe for children and adults with pulmonary hypertension and congenital heart defects (CHD). With iCMR, the calculation of pulmonary vascular resistance (PVR) in children with complex CHD with multilevel shunt lesions is accurate. In this paper, we describe the role of the MRI-guided right-sided cardiac catheterization procedure to accurately estimate PVR in the setting of multiple shunt lesions (ventricular septal defect and patent ductus arteriosus) and to address the clinical question of operability in an adolescent with trisomy 21 and severe pulmonary hypertension. Full article
(This article belongs to the Special Issue Focused Update on Pulmonary Hypertension in Children)
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11 pages, 8903 KiB  
Brief Report
Segmental Pulmonary Hypertension in Children with Congenital Heart Disease
by Bibhuti B. Das, Benjamin Frank and Dunbar Ivy
Medicina 2020, 56(10), 492; https://doi.org/10.3390/medicina56100492 - 24 Sep 2020
Cited by 1 | Viewed by 2447
Abstract
Segmental pulmonary hypertension is a complex condition in children that encompasses many congenital heart diseases including pulmonary atresia with ventricular septal defect, hemitruncus/truncus arteriosus with branch pulmonary artery stenosis, unilateral absent pulmonary artery, and several post-tricuspid shunt lesions. Multimodality imaging is required to [...] Read more.
Segmental pulmonary hypertension is a complex condition in children that encompasses many congenital heart diseases including pulmonary atresia with ventricular septal defect, hemitruncus/truncus arteriosus with branch pulmonary artery stenosis, unilateral absent pulmonary artery, and several post-tricuspid shunt lesions. Multimodality imaging is required to confirm and assess pulmonary vascular disease in subjects with major aorto-pulmonary collaterals. We describe 3 children with complex congenital heart defects who have a variable degree of segmental pulmonary hypertension and discuss management strategies and the role of interventional and/or pulmonary hypertension targeted therapies. Full article
(This article belongs to the Special Issue Focused Update on Pulmonary Hypertension in Children)
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