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Advances in the Diagnosis and Treatment of Pulmonary Vascular Diseases
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Advances in the Diagnosis and Treatment of Pulmonary Vascular Diseases

A special issue of Journal of Cardiovascular Development and Disease (ISSN 2308-3425). This special issue belongs to the section "Cardiovascular Clinical Research".

Deadline for manuscript submissions: closed (15 August 2023) | Viewed by 3545

Special Issue Editor

Dr. Grzegorz Kopec

E-Mail Website
Guest Editor
Pulmonary Circulation Centre, Department of Cardiac and Vascular Diseases, Jagiellonian University Medical College, John Paul II Hospital in Krakow ul, Pradnicka 80, 31-202 Krakow, Poland
Interests: pulmonary hypertension; chronic thromboembolic pulmonary disease
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

It is my privilege to invite you to this Special Issue of the Journal of Cardiovascular Development and Disease titled “Advances in Diagnosis and Treatment of Pulmonary Vascular Diseases”. We dedicate this issue mainly but not solely to pulmonary hypertension (PAH) and other diseases of pulmonary circulation.

The definition of pulmonary hypertension has been revised by recent guidelines of the European Society of Cardiology, but how this change can affect epidemiology of this disease, the approach to its management, and patients’ outcome are not well known. Therefore, in the present issue, we are going to prioritize studies which enroll patients with pulmonary hypertension diagnosed based on the novel definition.

The clinical characteristics of the PAH population have been changing in recent decades. Currently, a significant number of patients are more than 65 years old at the time of diagnosis and have several different diseases when PAH is diagnosed. Thus, of special interest is differential diagnosis between PAH and other forms of pulmonary hypertension, including those due to left heart diseases and due to diseases of the respiratory system. We are also interested in papers presenting how different comorbidities can affect patients’ outcome.

PAH is very rare in the pediatric population, and there is a paucity of data from clinical studies on the optimal therapy of PAH in this population. Therefore, epidemiological data on PAH in children, cohort studies analyzing clinical outcomes, as well as clinical trials in this field are within scope in the present issue of the Journal of Clinical Medicine.

Despite increasing knowledge on PAH mechanisms, the etiology of this disease is still not well understood. Accordingly, studies presenting new data from preclinical studies on the pathophysiology of human PAH, especially with use of the emerging -omics technologies, will be considered in the present issue.

Recently, we have also observed a revolution in the treatment modalities of other diseases of pulmonary circulation, including pulmonary embolism and congenital malformations. This Special Issue will thus accept clinically important studies in this field.

Dr. Grzegorz Kopec
Guest Editor

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. Journal of Cardiovascular Development and Disease 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 2700 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
  • pulmonary embolism
  • venous thromboembolic disease
  • pulmonary vascular disease
  • congenital heart disease

Published Papers (2 papers)

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17 pages, 1836 KiB  
Review
Unique Pulmonary Hypertensive Vascular Diseases Associated with Heart and Lung Developmental Defects
by Hidekazu Ishida, Jun Maeda, Keiko Uchida and Hiroyuki Yamagishi
J. Cardiovasc. Dev. Dis. 2023, 10(8), 333; https://doi.org/10.3390/jcdd10080333 - 3 Aug 2023
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Abstract
Although pediatric pulmonary hypertension (PH) shares features and mechanisms with adult PH, there are also some significant differences between the two conditions. Segmental PH is a unique pediatric subtype of PH with unclear and/or multifactorial pathophysiological mechanisms, and is often associated with complex [...] Read more.
Although pediatric pulmonary hypertension (PH) shares features and mechanisms with adult PH, there are also some significant differences between the two conditions. Segmental PH is a unique pediatric subtype of PH with unclear and/or multifactorial pathophysiological mechanisms, and is often associated with complex congenital heart disease (CHD), pulmonary atresia with ventricular septal defect, and aortopulmonary collateral arteries. Some cases of complex CHD, associated with a single ventricle after Fontan operation, show pathological changes in the small peripheral pulmonary arteries and pulmonary vascular resistance similar to those observed in pulmonary arterial hypertension (PAH). This condition is termed as the pediatric pulmonary hypertensive vascular disease (PPHVD). Recent advances in genetics have identified the genes responsible for PAH associated with developmental defects of the heart and lungs, such as TBX4 and SOX17. Targeted therapies for PAH have been developed; however, their effects on PH associated with developmental heart and lung defects remain to be established. Real-world data analyses on the anatomy, pathophysiology, genetics, and molecular biology of unique PPHVD cases associated with developmental defects of the heart and lungs, using nationwide and/or international registries, should be conducted in order to improve the treatments and prognosis of patients with these types of pediatric PH. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Treatment of Pulmonary Vascular Diseases)
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18 pages, 8941 KiB  
Case Report
Novel Hybrid Treatment for Pulmonary Arterial Hypertension with or without Eisenmenger Syndrome: Double Lung Transplantation with Simultaneous Endovascular or Classic Surgical Closure of the Patent Ductus Arteriosus (PDA)
by Tomasz Stącel, Paweł Sybila, Agata Mędrala, Marek Ochman, Magdalena Latos, Fryderyk Zawadzki, Anna Pióro, Piotr Pasek, Piotr Przybyłowski, Tomasz Hrapkowicz, Ewa Mroczek, Agnieszka Kuczaj, Grzegorz Kopeć, Roland Fiszer, Szymon Pawlak, Anita Stanjek-Cichoracka and Maciej Urlik
J. Cardiovasc. Dev. Dis. 2022, 9(12), 457; https://doi.org/10.3390/jcdd9120457 - 14 Dec 2022
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Abstract
Patients with pulmonary arterial hypertension (PAH) become candidates for lung or lung and heart transplantation when the maximum specific therapy is no longer effective. The most difficult challenge is choosing one of the above options in the event of symptoms of right ventricular [...] Read more.
Patients with pulmonary arterial hypertension (PAH) become candidates for lung or lung and heart transplantation when the maximum specific therapy is no longer effective. The most difficult challenge is choosing one of the above options in the event of symptoms of right ventricular failure. Here, we present two female patients with PAH: (1) a 21-year-old patient with Eisenmenger syndrome, caused by a congenital defect—patent ductus arteriosus (PDA); and (2) a 39-year-old patient with idiopathic PAH and coexistent PDA. Their common denominator is PDA and the hybrid surgery performed: double lung transplantation with simultaneous PDA closure. The operation was performed after pharmacological bridging (conditioning) to transplantation that lasted for 33 and 70 days, respectively. In both cases, PDA closure effectiveness was 100%. Both patients survived the operation (100%); however, patient no. 1 died on the 2nd postoperative day due to multi-organ failure; while patient no. 2 was discharged home in full health. The authors did not find a similar description of the operation in the available literature and PubMed database. Hence, we propose this new treatment method for its effectiveness and applicability proven in our practice. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Treatment of Pulmonary Vascular Diseases)
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