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State of the Art: Clinical Management of Pulmonary Hypertension
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State of the Art: Clinical Management of Pulmonary Hypertension

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Cardiovascular Medicine".

Deadline for manuscript submissions: closed (31 August 2023) | Viewed by 5928

Special Issue Editor

Dr. Shigefumi Fukui

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Guest Editor
Department of Cardiology, Fujita Health University, Toyoake, Japan
Interests: pulmonary hypertension; chronic thromboembolic pulmonary hypertension; pulmonary arterial hypertension; right heart function; cardiopulmonary comorbidity; lung disease

Special Issue Information

Dear Colleagues,

In the past couple of decades, the management of pulmonary hypertension (PH) has significantly evolved, especially in the field of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Briefly, early diagnosis, PH-specific therapies, and initial combination therapy for PAH has improved prognosis, as well as multimodal imaging, invasive intervention such as pulmonary endarterectomy and balloon pulmonary angioplasty, and PH-specific therapies for CTEPH. However, there are still significant issues to be clarified. Although right ventricular function is considered to be an important prognostic predictor of PH, its adaptation for increased afterload varies among patients and underlying diseases. The treatment algorithm for patients with idiopathic, heritable, drug-associated, and connective tissue-disease-associated PAH in the latest ESC/ERS PH guidelines differentiates initial combination therapy and monotherapy according to the presence of cardiopulmonary comorbidities. PH-specific therapies for patients with cardiopulmonary comorbidities, called “atypical PAH”, are sometimes challenging because of increased adverse effects and limited efficacy. Finally, the management of PH associated with lung diseases and/or hypoxia (Group 3) is also challenging, including which patients we need to treat with PH-specific therapies and which drugs we need to select. In this Special Issue, we would mainly like to highlight those unsolved issues in the management of PH.

Dr. Shigefumi Fukui
Guest Editor

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Keywords

  • pulmonary arterial hypertension
  • chronic thromboembolic pulmonary hypertension
  • multimodal imaging
  • pulmonary endarterectomy
  • balloon pulmonary angioplasty

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Published Papers (3 papers)

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13 pages, 8084 KiB  
Article
Evidence for Hypoxia-Induced Shift in ATP Production from Glycolysis to Mitochondrial Respiration in Pulmonary Artery Smooth Muscle Cells in Pulmonary Arterial Hypertension
by Satoshi Akagi, Kazufumi Nakamura, Megumi Kondo, Satoshi Hirohata, Heiichiro Udono, Mikako Nishida, Yukihiro Saito, Masashi Yoshida, Toru Miyoshi and Hiroshi Ito
J. Clin. Med. 2023, 12(15), 5028; https://doi.org/10.3390/jcm12155028 - 31 Jul 2023
Cited by 5 | Viewed by 2042
Abstract
Background: The metabolic state of pulmonary artery smooth muscle cells (PASMCs) from patients with pulmonary arterial hypertension (PAH) is not well understood. In this study, we examined the balance between glycolysis and mitochondrial respiration in non-PAH-PASMCs and PAH-PASMCs under normoxia and hypoxia. Methods: [...] Read more.
Background: The metabolic state of pulmonary artery smooth muscle cells (PASMCs) from patients with pulmonary arterial hypertension (PAH) is not well understood. In this study, we examined the balance between glycolysis and mitochondrial respiration in non-PAH-PASMCs and PAH-PASMCs under normoxia and hypoxia. Methods: We investigated the enzymes involved in glycolysis and mitochondrial respiration, and studied the two major energy-yielding pathways (glycolysis and mitochondrial respiration) by measuring extracellular acidification rate (ECAR) and cellular oxygen consumption rate (OCR) using the Seahorse extracellular flux technology. Results: Under both normoxia and hypoxia, the mRNA and protein levels of pyruvate dehydrogenase kinase 1 and pyruvate dehydrogenase were increased in PAH-PASMCs compared with non-PAH-PASMCs. The mRNA and protein levels of lactate dehydrogenase, as well as the intracellular lactate concentration, were also increased in PAH-PASMCs compared with non-PAH-PASMCs under normoxia. However, these were not significantly increased in PAH-PASMCs compared with non-PAH-PASMCs under hypoxia. Under normoxia, ATP production was significantly lower in PAH-PASMCs (59 ± 5 pmol/min) than in non-PAH-PASMCs (70 ± 10 pmol/min). On the other hand, ATP production was significantly higher in PAH-PASMCs (31 ± 5 pmol/min) than in non-PAH-PASMCs (14 ± 3 pmol/min) under hypoxia. Conclusions: There is an underlying change in the metabolic strategy to generate ATP production under the challenge of hypoxia. Full article
(This article belongs to the Special Issue State of the Art: Clinical Management of Pulmonary Hypertension)
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12 pages, 534 KiB  
Article
Pulmonary Arterial Hypertension Associated with Portal Hypertension and HIV Infection: Comparative Characteristics and Prognostic Predictors
by Fabio Dardi, Daniele Guarino, Francesco Cennerazzo, Alberto Ballerini, Ilenia Magnani, Riccardo Bertozzi, Federico Donato, Giulia Martini, Alessandra Manes, Nazzareno Galiè and Massimiliano Palazzini
J. Clin. Med. 2023, 12(10), 3425; https://doi.org/10.3390/jcm12103425 - 12 May 2023
Cited by 1 | Viewed by 1781
Abstract
Background: Pulmonary arterial hypertension (PAH) may complicate both portal hypertension (Po-PAH) and HIV infection (HIV-PAH). These two conditions, however, frequently coexist in the same patient (HIV/Po-PAH). We evaluated clinical, functional, hemodynamic characteristics and prognostic parameters of these three groups of patients. Methods: We [...] Read more.
Background: Pulmonary arterial hypertension (PAH) may complicate both portal hypertension (Po-PAH) and HIV infection (HIV-PAH). These two conditions, however, frequently coexist in the same patient (HIV/Po-PAH). We evaluated clinical, functional, hemodynamic characteristics and prognostic parameters of these three groups of patients. Methods: We included patients with Po-PAH, HIV-PAH and HIV/Po-PAH referred to a single center. We compared clinical, functional and hemodynamic parameters, severity of liver disease [Child–Turcotte–Pugh (CTP) and Model for End-stage Liver Disease-Na (MELD-Na) scores], CD4 count and highly active antiretroviral therapy (HAART) administration. Prognostic variables were identified through Cox-regression analysis. Results: Patients with Po-PAH (n = 128) were the oldest, patients with HIV-PAH (n = 41) had the worst hemodynamic profile and patients with HIV/Po-PAH (n = 35) had the best exercise capacity. Independent predictors of mortality were age and CTP score for Po-PAH, HAART administration for HIV-PAH, MELD-Na score and hepatic venous-portal gradient for HIV/Po-PAH. Conclusions: Patients with HIV/Po-PAH are younger and have a better exercise capacity than patients with Po-PAH, have a better exercise capacity and hemodynamic profile compared to patients with HIV-PAH, and their prognosis seems to be related to the hepatic disease rather than to HIV infection. The prognosis of patients with Po-PAH and HIV-PAH seems to be related to the underlying disease. Full article
(This article belongs to the Special Issue State of the Art: Clinical Management of Pulmonary Hypertension)
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8 pages, 2473 KiB  
Case Report
A Case of Giant Goiter Associated with Airway Stenosis Caused by Long-Term Intravenous Epoprostenol Therapy for Idiopathic Pulmonary Arterial Hypertension
by Kazuto Nishiura, Kazuhiko Nakazato, Tetsuro Yokokawa, Yoshinori Suzuki, Yuta Kurosawa, Kento Wada, Takeshi Shimizu, Masayoshi Oikawa, Atsushi Kobayashi, Koichi Sugimoto, Norshalena Shakespear, Yuko Hashimoto and Yasuchika Takeishi
J. Clin. Med. 2023, 12(19), 6359; https://doi.org/10.3390/jcm12196359 - 4 Oct 2023
Cited by 2 | Viewed by 1595
Abstract
Idiopathic pulmonary arterial hypertension is a progressive and life-threatening disease with pulmonary vasculature remodeling, leading to right-sided heart failure. Epoprostenol (prostaglandin I2) is highly recommended for patients with severe pulmonary arterial hypertension (PAH) categorized by the World Health Organization as functional [...] Read more.
Idiopathic pulmonary arterial hypertension is a progressive and life-threatening disease with pulmonary vasculature remodeling, leading to right-sided heart failure. Epoprostenol (prostaglandin I2) is highly recommended for patients with severe pulmonary arterial hypertension (PAH) categorized by the World Health Organization as functional class III or IV. It has been reported that prostaglandin I2 analogs can cause thyroid gland swelling and abnormal thyroid function. A 34-year-old woman was diagnosed with idiopathic pulmonary arterial hypertension and started receiving continuous intravenous epoprostenol. Three years after starting epoprostenol, she began complaining of neck swelling and was diagnosed with Graves’ disease. The patient’s thyroid function was controlled by thiamazole and levothyroxine; nevertheless, her thyroid gland enlargement worsened as the epoprostenol dose was titrated. After 20 years, she developed respiratory failure with a giant goiter leading to airway stenosis, and she passed away. The pathological autopsy confirmed a massive goiter associated with hyperthyroidism and airway stenosis. We experienced a case of idiopathic pulmonary hypertension with a giant goiter and airway stenosis after long-term intravenous epoprostenol therapy. Full article
(This article belongs to the Special Issue State of the Art: Clinical Management of Pulmonary Hypertension)
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