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Life
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Pulmonary Hypertension: Insights on Pathophysiology, Diagnosis and Management
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Pulmonary Hypertension: Insights on Pathophysiology, Diagnosis and Management

A special issue of Life (ISSN 2075-1729). This special issue belongs to the section "Physiology and Pathology".

Deadline for manuscript submissions: closed (31 July 2024) | Viewed by 7121

Special Issue Editor

Dr. Alexandra Arvanitaki

E-Mail Website
Guest Editor
1. First Department of Cardiology, AHEPA University Hospital, School of Medicine, Aristotle University of Thessaloniki, Thessaloniki St. Kiriakidi 1, 54642 Thessaloniki, Greece
2. Pulmonary Hypertension Unit, Royal Brompton Hospital, Guy’s and St Thomas’ Foundation Trust, Sydney Street, London, UK
Interests: pulmonary hypertension; adult congenital heart disease; heart failure; thrombosis
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Pulmonary hypertension (PH) represents a rare and heterogeneous group of pulmonary vasculopathies defined by elevated mean pulmonary arterial pressure and pulmonary vascular resistance, which progressively lead to right heart failure and premature death if not diagnosed early and promptly treated. In the past 20 years there has been increased research interest in the fields of pathophysiology and targeted pulmonary arterial hypertension (PAH) therapy, while new diagnostic modalities and algorithms were employed to detect PH early. In addition, national and international registries have provided important information about the epidemiology of the disease, which changes steadily over time. Whereas younger female patients were diagnosed with idiopathic PAH in 1980, it is now frequently diagnosed in elderly patients with comorbidities, while a female predominance is not the rule among older patients. Furthermore, patients’ risk stratification according to clinical, neurohormonal, echocardiographic, and hemodynamic parameters plays an important role in decisions on therapeutic management. Finally, survival has substantially increased over time, but it is still poor in several PH subgroups.

In this Special Issue, we would like to invite original clinical as well as basic research and state-of-the-art reviews related to pathophysiology, diagnostics, and therapeutics in the whole spectrum of PH. We would be grateful to receive your submissions to move the field of PH forward.

Dr. Alexandra Arvanitaki
Guest Editor

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Keywords

  • pulmonary hypertension
  • pulmonary arterial hypertension
  • pathophysiology
  • diagnosis
  • risk stratification
  • management
  • pharmacotherapy

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Published Papers (6 papers)

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Research

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12 pages, 613 KiB  
Article
Haemolytic Anaemia-Related Pulmonary Hypertension
by Panagiotis Karyofyllis, Eftychia Demerouti, Eleftheria-Garyfallia Tsetika, Styliani Apostolopoulou, Panagiotis Tsiapras, Ioannis Iakovou and Dimitrios Tsiapras
Life 2024, 14(7), 876; https://doi.org/10.3390/life14070876 - 14 Jul 2024
Viewed by 502
Abstract
Haemolytic anaemia represents a risk factor for the development of pulmonary hypertension (PH), currently classified as World Health Organization group 5 PH, and data regarding appropriate therapeutic strategy are limited. A total of 28 patients, 85.7% with thalassaemia and 14.3% with sickle cell [...] Read more.
Haemolytic anaemia represents a risk factor for the development of pulmonary hypertension (PH), currently classified as World Health Organization group 5 PH, and data regarding appropriate therapeutic strategy are limited. A total of 28 patients, 85.7% with thalassaemia and 14.3% with sickle cell disease, with a diagnosis of PH confirmed by right heart catheterization were included in the study. The patients were divided into three groups according to the PH haemodynamic definition and overall diagnostic approach: 42.9% had precapillary PH (pulmonary arterial hypertension—PAH group), 25% had post-capillary PH, and 32.1% had chronic thromboembolic PH (CTEPH) (29% of b-thalassemia and 50% of SCD patients). The therapeutic approach in each group and its impact on the outcome and haemodynamics were recorded. PAH-specific drug therapy received 82.1% of patients, and balloon pulmonary angioplasty (BPA) was performed in six patients with CTEPH. There were statistically significant differences in baseline mPAP and PVR values between the CTEPH-haemolytic anaemia group and other groups. PAH-specific drug therapy resulted in haemodynamic improvement for the PAH group. Patients who underwent BPA had improved pulmonary haemodynamics. The median survival time was 162 months, and the survival rate was 1 year—100%; 2, 3, 4, 5, and 6 years—96%; 9 years—90%; and 13 years—78%. In patients with haemolytic anaemia, the wide spectrum of induced PH highlighted the importance of a correct predominant diagnosis. BPA in CTEPH patients and specific-PAH drug therapy for PAH patients represent potential therapeutic strategies; however, the management should be offered in expert PH centres under individualized approaches for patients. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Insights on Pathophysiology, Diagnosis and Management)
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22 pages, 3499 KiB  
Article
Elderly Patients with Idiopathic Pulmonary Hypertension: Clinical Characteristics, Survival, and Risk Stratification in a Single-Center Prospective Registry
by Natalia Goncharova, Kirill Lapshin, Aelita Berezina, Maria Simakova, Alexandr Marichev, Irina Zlobina, Narek Marukyan, Kirill Malikov, Alexandra Aseeva, Vadim Zaitsev and Olga Moiseeva
Life 2024, 14(2), 259; https://doi.org/10.3390/life14020259 - 16 Feb 2024
Viewed by 1012
Abstract
Introduction: The predictive value of the risk stratification scales in elderly patients with IPAH might differ from that in younger patients. It is unknown whether young and older IPAH patients have the same survival dependence on PAH-specific therapy numbers. The aim of this [...] Read more.
Introduction: The predictive value of the risk stratification scales in elderly patients with IPAH might differ from that in younger patients. It is unknown whether young and older IPAH patients have the same survival dependence on PAH-specific therapy numbers. The aim of this study was to evaluate the prognostic relevance of risk stratification scales and PAH medication numbers in elderly IPAH patients in comparison with young IPAH patients. Materials and methods: A total of 119 patients from a prospective single-center PAH registry were divided into group I < 60 years old (n = 89) and group II ≥ 60 years old (n = 30). ESC/ERS, REVEAL, and REVEAL 2.0 risk stratification scores were assessed at baseline, as well as H2FpEF score and survival at follow-up. Results: During a mean follow-up period of 2.9 years (1.63; 6.0), 42 (35.3%) patients died; at 1, 2, 3, 5, 7, and 10 years, survival was 95%, 88.6%, 78.5%, 61.7%, 48.5%, and 33.7%, respectively. No survival differences were observed between the two groups, despite the use of monotherapy in the elderly patients. The best predictive REVEAL value in elderly patients (IPAH patients ≥ 60 years) was AUC 0.73 (0.56–0.91), p = 0.03; and in patients with LHD comorbidities in the entire cohort, it was AUC 0.73 (0.59–0.87), p < 0.009. Factors independently associated with death in the entire cohort were CKD (p = 0.01, HR 0.2), the right-to-left ventricle dimension ratio (p = 0.0047, HR 5.97), and NT-proBNP > 1400 pg/mL (p = 0.008, HR 3.18). Conclusion: Risk stratification in the elderly IPAH patients requires a fundamentally different approach than that of younger patients, taking into account the initial limitations in physical performance and comorbidities that interfere with current assessment scores. The REVEAL score reliably stratifies patients at any age and LHD comorbidities. The initial monotherapy seems to be reasonable in patients over 60 years. Selection tools for initial combination PAH therapy in older IPAH patients with comorbidities need to be validated in prospective observational studies. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Insights on Pathophysiology, Diagnosis and Management)
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13 pages, 2243 KiB  
Article
Temporal Trends in Diagnostic Hemodynamics and Survival of Patients with Pulmonary Hypertension: A Single-Center Study
by Georgios E. Papadopoulos, Alexandra Arvanitaki, Eleftherios Markidis, Sophia Anastasia Mouratoglou, Ioannis T. Farmakis, Panagiotis Gourgiotis, Thomas Chrysochoidis Trantas, Christos Feloukidis, Antonios Kouparanis, Matthaios Didagelos, Vasilis Grosomanidis, Antonios Ziakas and George Giannakoulas
Life 2023, 13(11), 2225; https://doi.org/10.3390/life13112225 - 19 Nov 2023
Cited by 1 | Viewed by 1790
Abstract
Advances in the diagnosis and treatment of pulmonary hypertension (PH) have gradually improved the disease course. This retrospective cohort study aims to explore the diagnostic hemodynamic profile and survival of PH patients and their temporal changes, as well as investigate potential prognostic factors. [...] Read more.
Advances in the diagnosis and treatment of pulmonary hypertension (PH) have gradually improved the disease course. This retrospective cohort study aims to explore the diagnostic hemodynamic profile and survival of PH patients and their temporal changes, as well as investigate potential prognostic factors. Overall, 257 adult patients were diagnosed with PH following right heart catheterization (RHC) from January 2008 to June 2023 according to the hemodynamic cut-off values proposed by the corresponding ESC/ERS guidelines at the time RHC was performed. Of these patients, 46.3% were Group 1, 17.8% Group 2, 14.0% Group 3, 18.0% Group 4, and 3.0% Group 5 PH. Temporal improvement in both diagnostic hemodynamic profile and survival of patients with PH and pulmonary arterial hypertension (PAH) was identified after 2013. Survival analysis demonstrated 5-year survival rates of 65% in Group 1 PH (90.3% in idiopathic PAH) and 77% in Group 4 PH. PAH patients being at low risk at diagnosis presented a similar 1-year all-cause mortality rate (12.4%) with high-risk ones (12.8%), primarily due to non-PH-related causes of death (62%), while high-risk patients died mostly due to PH (67%). The observed improvements in diagnostic hemodynamic profiles and overall survival highlight the importance of timely diagnosis and successful treatment strategies in PH. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Insights on Pathophysiology, Diagnosis and Management)
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Review

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13 pages, 6084 KiB  
Review
Northern Pulmonary Hypertension: A Forgotten Kind of Pulmonary Circulation Pathology
by Djuro Kosanovic, Sergey N. Avdeev, Andrey P. Milovanov and Andrey L. Chernyaev
Life 2024, 14(7), 875; https://doi.org/10.3390/life14070875 - 13 Jul 2024
Viewed by 394
Abstract
Northern pulmonary hypertension (NPH) is a medical condition that is still enigmatic in non-Russian-speaking countries. The extant previous literature is mostly available in the Russian language and, therefore, not accessible to the rest of the world. The recent increased interest in climate changes [...] Read more.
Northern pulmonary hypertension (NPH) is a medical condition that is still enigmatic in non-Russian-speaking countries. The extant previous literature is mostly available in the Russian language and, therefore, not accessible to the rest of the world. The recent increased interest in climate changes and environmental effects on pulmonary circulation prompted us to summarize the knowledge from the past about the effects of cold on pulmonary vasculature. In this review, we, for the first time, describe, in detail, the pathological attributes of human NPH, a medical disorder that occurs in people living in extremely cold regions, in the English language. Briefly, NPH is characterized by the hyper-muscularization of the pulmonary arteries and de novo muscularization of the arterioles with the ultimate development of right ventricular hypertrophy. However, the profound molecular mechanisms of the NPH pathology are to be revealed in future comprehensive studies. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Insights on Pathophysiology, Diagnosis and Management)
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10 pages, 548 KiB  
Review
Pulmonary Hypertension and Hypothyroidism—Still an Important Clinical Coincidence in Paediatric Population, an Endocrinologist’s Point of View
by Agnieszka Lecka-Ambroziak and Karolina Kot
Life 2024, 14(3), 302; https://doi.org/10.3390/life14030302 - 26 Feb 2024
Viewed by 1240
Abstract
There is limited data on hypotheses linking autoimmune thyroid diseases (AITD) and hypothyroidism with pulmonary hypertension (PH). Moreover, the prevalence of this coincidence, as well as the possible common pathogenic mechanisms, are even less explicit in paediatric population. We present a review of [...] Read more.
There is limited data on hypotheses linking autoimmune thyroid diseases (AITD) and hypothyroidism with pulmonary hypertension (PH). Moreover, the prevalence of this coincidence, as well as the possible common pathogenic mechanisms, are even less explicit in paediatric population. We present a review of recently published articles regarding relatively large cohorts of children with PH, coming from paediatric PH registries, aiming to clarify the coincidence of PH and AITD, especially hypothyroidism, and discuss its possible mutual impact. Although thyroid disorders have been excluded from the latest PH classification, it is still important to remember the possibility of this coincidence as it may significantly influence patients’ clinical outcome. Moreover, children with PH may need multidisciplinary care due to the relatively frequent coexistence with not only hormonal abnormalities but also growth impairment, genetic disorders, and mental delay. Further specific paediatric studies are needed to improve the care in this rare disease, especially in patients with other comorbidities present. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Insights on Pathophysiology, Diagnosis and Management)
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Other

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7 pages, 1059 KiB  
Case Report
Familiar Disposition of May–Thurner Syndrome—A Case Series
by Stefanie Nowak, André Jakob, Robert Dalla Pozza, Sebastian Michel, Nikolaus A. Haas and Joseph Pattathu
Life 2024, 14(2), 221; https://doi.org/10.3390/life14020221 - 4 Feb 2024
Cited by 1 | Viewed by 1240
Abstract
May–Thurner syndrome is a venous compression syndrome of the pelvic vessels that represents a relevant risk factor for thrombus formation. The standard procedure to secure a diagnosis is venography, followed by endovascular therapy as the preferred treatment choice if the patient is symptomatic. [...] Read more.
May–Thurner syndrome is a venous compression syndrome of the pelvic vessels that represents a relevant risk factor for thrombus formation. The standard procedure to secure a diagnosis is venography, followed by endovascular therapy as the preferred treatment choice if the patient is symptomatic. In our case series, there are three related patients with May–Thurner syndrome. A 16-year-old female was admitted with pulmonary embolism, dyspnoea and hip pain. The compression syndrome was diagnosed with interventional venography, and the patient received venous stent implantation. Due to her family history, we also suspected her mother to be affected by the syndrome and elucidated the diagnosis shortly afterwards by invasive venography. Subsequently, we examined the patient’s 19-year-old brother, and magnetic resonance imaging confirmed May–Thurner syndrome. A similar case series has not been published before. In this case, the family relation indicates a possible hereditary aspect of May–Thurner syndrome. This hypothesis should be the subject of further research. In conclusion, it is essential to assess family history thoroughly when treating patients with May–Thurner syndrome. Full article
(This article belongs to the Special Issue Pulmonary Hypertension: Insights on Pathophysiology, Diagnosis and Management)
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