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Porifera are considered key components of benthic communities, being typical inhabitants of both shallow and deep waters, from the mesolittoral to the bathyal zone. Although sponges are one of the most common organisms in Western Mediterranean ecosystems, in many areas the knowledge on this taxon is still very limited. In the current study, we aim to analyse and compare the sponge communities characterising the hard bottoms of two islands, Montecristo and Giglio, belonging to the Tuscan Archipelago National Park (TANP, Italy), in relation to a bathymetric gradient. A total of 340 samples were collected, 13 orders and 48 species (46 Demospongiae and 2 Calcarea) identified, with Chondrosia reniformis representing the most recorded species, being found at all sites at almost all depths. Depth was the only significant factor in the observed pattern, while the sponge community is shared between the two Islands. In addition, our data were analysed together with data available in the literature on sponges of the Tuscan Archipelago, to assess the sampling effort. Among all the Islands of the Archipelago, Giglio and Montecristo showed the higher mean species diversity. Nonetheless, the sampling effort resulted not sufficient for each Island and future studies targeting Porifera are needed. Our work is the first summarising the sponge diversity of the entire Tuscan Archipelago, and one of the few focussing entirely on the sponge fauna. These results provide new important information on the area of the TANP, aimed to become a future Marine Protected Area, adding new records, and highlighting the high diversity of a poorly known area.

Objectives: To investigate clinical characteristics, symptom profile, testing practices, treatment patterns and quality of life (QoL) among patients with pulmonary arterial hypertension (PAH) in Latin America., Design: Data from the Adelphi Real World PAH Disease Specific Programme, a cross-sectional survey with retrospective data collection., Setting: University/teaching hospital, regional centres, private practices and government institutions in Argentina, Brazil, Colombia and Mexico., Participants: 246 physicians provided data for 958 patients, of which 533 patients also self-reported data., Results: Mean (SD) patient age was 53.7 (17) years, 70% of patients were female and 79% were WHO functional class (WHO FC) I-II. Overall, 76% had undergone a right heart catheterisation, ranging from 92% in Argentina to 64% in Brazil (p<0.0001). Only 28% underwent a simplified risk assessment strategy in the past 12 months, ranging from 46% in Argentina to 16% in Brazil. Fatigue and dyspnoea on exertion were reported most commonly by physicians (37% and 53%) and patients (68% and 67%). Patient-physician agreement on symptom reporting was minimal-to-weak (kappa, 0.21-0.42). PAH-specific combination therapy varied across countries (21% Mexico, 30% Brazil, 70% Colombia and 79% Argentina, p<0.0001)). Overall, 73% of patients received a phosphodiesterase type 5 inhibitor; 52% an endothelin receptor antagonist, 15% a prostacyclin pathway agent and 11% a soluble guanylate cyclase stimulator. The mean (SD) EQ-5D (generic instrument to define quality of life)utility ranged from 0.66 (0.20) to 0.70 (0.20) across countries and the mean (SD) EQ-5D Visual Analogue Scale (VAS) was 67.0 (18.10). Lower VAS and utility scores were reported among patients with higher WHO FC (p<0.05)., Conclusions: Patients reported a high burden of PAH in terms of symptoms and QoL, particularly within higher WHO FC. Low usage of risk assessment strategies and PAH-specific combination therapy was seen in Brazil and Mexico. Further research could identify barriers to prescribing optimal treatment., Competing Interests: Competing interests: TPi is an employee of Janssen and is a Janssen shareholder. MS and JH are employees of Adelphi Real World. MO-L, RS, IMB, JLHO, AL, GM, AR, AV and TPu received consulting fees from Janssen., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ Group.)

Care of pulmonary hypertension (PH) patients in special situations requires insightful knowledge of the pathophysiology of the cardiopulmonary system and close interaction with different specialists, depending on the situation. The role of this task force was to gather knowledge about five conditions that PH patients may be faced with. These conditions are 1) perioperative care; 2) management of pregnancy; 3) medication adherence; 4) palliative care; and 5) the influence of climate on PH. Many of these aspects have not been covered by previous World Symposia on Pulmonary Hypertension. All of the above conditions are highly affected by psychological, geographical and socioeconomic factors, and share the need for adequate healthcare provision. The task force identified significant gaps in information and research in these areas. The current recommendations are based on detailed literature search and expert opinion. The task force calls for further studies and research to better understand and address the special circumstances that PH patients may encounter., Competing Interests: Conflict of interest: I.R. Preston reports grants from Janssen, Merck, United Therapeutics and Respira, consultancy fees from Janssen, Merck, United Therapeutics, Liquidia, Gossamer and Aerovate, payment or honoraria for lectures, presentations, manuscript writing or educational events from Janssen and United Therapeutics, and support for attending meetings from Janssen and United Therapeutics. L.S. Howard reports grants from MSD, consultancy fees from Ferrer, Janssen and Morphic, payment or honoraria for lectures, presentations, manuscript writing or educational events from Janssen, MSD, Aerovate and Ferrer, payment for expert testimony from Janssen, support for attending meetings from Janssen and Gossamer Bio, participation on a data safety monitoring board or advisory board with MSD, Janssen, Gossamer Bio, Apollo Therapeutics and Altavant, and stock (or stock options) with Circular, ATXA Therapeutics, iOWNA, Calibre Biometrics and OneWelbeck Clinic. D. Langleben reports grants from Acceleron/Merck, Aerovate and Janssen, consultancy fees from Merck, Janssen, Actelion, Enzyvant, PhaseBio, Gossamer Bio and Bayer, payment or honoraria for lectures, presentations, manuscript writing or educational events from Merck, Janssen and Bayer, support for attending meetings from Acceleron/Merck, Janssen, Enzyvant and Gossamer Bio, and participation on a data safety monitoring board or advisory board with Phase Bio, Merck, Janssen, Actelion, Enzyvant and Gossamer Bio. M. Lichtblau reports payment or honoraria for lectures, presentations, manuscript writing or educational events from, and participation on a data safety monitoring board or advisory board with MSD, and support for attending meetings from MSD and Janssen. T. Pulido reports grants from Aerovate, Bayer, Janssen, MSD, Pfizer and United Therapeutics, consultancy fees from Bayer, MSD, Gossamer Bio, Aerovate and Ferrer, payment or honoraria for lectures, presentations, manuscript writing or educational events from Bayer, Janssen, MSN Labs, Tuteur and Ferrer, support for attending meetings from Bayer, Janssen and Ferrer, participation on a data safety monitoring board or advisory board with Janssen, MSD, Bayer and Gossamer Bio, and is consultant and part of the advisory board for the Mexican Society of Pulmonary Arterial Hypertension. R. Souza reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Bayer and Janssen, and participation on a data safety monitoring board or advisory board with Janssen, Bayer and MSD. K.M. Olsson reports grants from Acceleron, MSD and Janssen, consultancy fees from Acceleron, Actelion, Janssen, Ferrer, Merck, AOP Health, Gossamer and Bayer, payment or honoraria for lectures, presentations, manuscript writing or educational events from Acceleron, Actelion, AOP Health, Janssen, Bayer, MSD, Ferrer and Gossamer, and support for attending meetings from Acceleron/MSD and Janssen/Actelion., (Copyright ©The authors 2024.)

Coastal areas conservation strategies often left deeper habitats, such as mesophotic ones, unprotected and exposed to anthropogenic activities. In this context, an approach for including the mesophotic zone inside protection plans is proposed, considering 27 Italian Marine Protected Areas (MPAs) as a model. MPAs were classified considering their bathymetries, exposure to marine heat waves (MHWs), mass mortality events (MMEs) and, using a local ecological knowledge (LEK) approach, the estimated resilience of certain sessile species after MMEs. Only 8 MPAs contained considerable mesophotic areas, with stronger MHWs mainly occurring in shallower MPAs, and MMEs mostly affecting coralligenous assemblages. Even with only a 10% response rate, the LEK approach provided useful information on the resilience of certain species, allowing us to suggest that the presence of nearby mesophotic areas can help shallower habitats facing climate change, thus making the "deep refugia" hypothesis, usually related to tropical habitats, applicable also for the Mediterranean Sea., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Carlo Cerrano reports financial support was provided by European Commission. Joaquim Garrabou reports financial support was provided by EU Framework Programme for Research and Innovation Euratom. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Marine ecosystems are facing a dramatic loss of biodiversity worldwide, together with a widespread collapse of habitats and their functionality. In this context, Marine Citizen Science (MCS) can be a powerful tool to monitor these changes over time. The flowering of very well-structured international projects is strengthening the scientific credibility of MCS data, especially when data are collected after specifically designed training programs and shared in public user-friendly repositories. Here we present a new perspective on the use of MCS in the Mediterranean area, along with the main benefits for the stakeholders (i.e., diving centers, trainers, and policymakers) and the users (i.e., divers), resumed in three pillars: Pillar I - MCS as a tool for the site valorization; Pillar II - MCS as a new career opportunity for graduated students; Pillar III - MCS as a business opportunity for diving centers. In the frame of the Quintuple Helix Approach, for which there is a strong need of a socioecological transition of the society and economy, we show how MCS can be a win-win-win solution for all the actors involved, providing the vision for new and highly qualified job and business opportunities for the diving sector., (© 2024. The Author(s).)

Cnidarians may dominate benthic communities, as in the case of coral reefs that foster biodiversity and provide important ecosystem services. Polyps may feed by predating mesozooplantkon and large motile prey, but many species further obtain autotrophic nutrients from photosymbiosis. Anthropogenic disturbance, such as the rise of seawater temperature and turbidity, can lead to the loss of symbionts, causing bleaching. Prolonged periods of bleaching can induce mortality events over vast areas. Heterotrophy may allow bleached cnidarians to survive for long periods of time. We tested the reinforcement of heterotrophic feeding of bleached polyps of Exaiptasia diaphana fed with both small zooplantkon and large prey, in order to evaluate if heterotrophy allows this species to compensate the reduction of autotrophy. Conversely to expected, heterotrophy was higher in unbleached polyps (+54% mesozooplankton prey and +11% large prey). The increase of heterotrophic intake may not be always used as a strategy to compensate autotrophic depletion in bleached polyps. Such a resilience strategy might be more species-specific than expected., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

This study investigated the relationship between Metabolic Syndrome (MetS), sleep disorders, the consumption of some nutrients, and social development factors, focusing on gender differences in an unbalanced dataset from a Mexico City cohort. We used data balancing techniques like SMOTE and ADASYN after employing machine learning models like random forest and RPART to predict MetS. Random forest excelled, achieving significant, balanced accuracy, indicating its robustness in predicting MetS and achieving a balanced accuracy of approximately 87%. Key predictors for men included body mass index and family history of gout, while waist circumference and glucose levels were most significant for women. In relation to diet, sleep quality, and social development, metabolic syndrome in men was associated with high lactose and carbohydrate intake, educational lag, living with a partner without marrying, and lack of durable goods, whereas in women, best predictors in these dimensions include protein, fructose, and cholesterol intake, copper metabolites, snoring, sobbing, drowsiness, sanitary adequacy, and anxiety. These findings underscore the need for personalized approaches in managing MetS and point to a promising direction for future research into the interplay between social factors, sleep disorders, and metabolic health, which mainly depend on nutrient consumption by region.

This manuscript on real-world evidence (RWE) in pulmonary hypertension (PH) incorporates the broad experience of members of the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative Real-World Evidence Working Group. We aim to strengthen the research community's understanding of RWE in PH to facilitate clinical research advances and ultimately improve patient care. Herein, we review real-world data (RWD) sources, discuss challenges and opportunities when using RWD sources to study PH populations, and identify resources needed to support the generation of meaningful RWE for the global PH community., Competing Interests: Boucly reports consulting fees from AOP Orphan; payment, honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from AOP Orphan, MSD, and Janssen; and support for attending meetings/travel from MSD and Janssen. Moles reports grants or contracts from Acceleron Pharma, Janssen, and CVS Caremark; and leadership or fiduciary role in other board, society, committee, or advocacy group, paid or unpaid, for Gossamer Bio. Elwing reports grants or contracts from United Therapeutics Corporation, Gossamer Bio, Bayer, Acceleron/Merck, Altavant, Aerovate, Tenax, Pharmosa, Actelion/Janssen, Arena, Lung, Liquidia, and Phase Bio; consulting fees for United Therapeutics Corporation; support for attending meetings and/or travel for United Therapeutics, Altavant, Aerovate, Bayer, Gossamer Bio, Liquida, Acceleron/Merck, Janssen/Actelion, and Insmed; and other financial or nonfinancial interests from Bayer as a grant reviewer. Jose reports grants or contracts from Team Phenomenal Hope Charity, Francis Family Foundation, United Therapeutics, and Janssen/Johnson & Johnson. Alves Jr. reports payment or honoraria from Bayer and Janssen; and support for attending meetings and/or travel from Janssen. Minty reports consulting fees from Janssen Pharmaceuticals and Orpyx Medical Technologies; and stock or stock options from Orpyx Medical Technologies. Weatherald reports grants or contracts from Janssen, Bayer, Merck, Canadian Institutes for Health Research, and the Heart & Stroke Foundation of Canada; consulting fees from Janssen and Merck; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Janssen and Merck; payment for expert testimony from Sprigings Intellectual Property Law; support for attending meetings and/or travel from Janssen; participation on a data safety monitoring board or advisory board from Janssen, Acceleron, and Université Laval; and leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid, from the Pulmonary Hypertension Association of Canada. Mathai reports grants or contracts from NIH/NHLBI (HL125175) and DOD (W81XWH‐20‐1‐0768); consulting fees from Actelion/Janssen, Aerovate, Merck, United Therapeutics Corporation, and Theravance; and participation on a data safety monitoring board or advisory board from Bayer. Sahay reports grants or contracts from United Therapeutics Corporation, Janssen, Bayer, Merck, Liquidia, and Gossamer; consulting fees from Merck, Liquidia, and Gossamer; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from United Therapeutics Corporation (received by hospital rather than self); patents planned, issued, or pending with Janssen; and participation on a data safety monitoring board or advisory board with GSK, Bayer, United Therapeutics, and Janssen. Pulido reports grants or contracts from Janssen, MSD, and United Therapeutics; consulting fees from Janssen, Bayer, Gossamer, and MSD; and payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Bayer, Janssen, and Pfizer. Badagliacca reports grants or contracts from United Therapeutics Corporation, Ferrer, and Janssen; consulting fees from MSD, United Therapeutics Corporation, Ferrer, Janssen, and AOP; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from MSD, United Therapeutics Corporation, Ferrer, Janssen, and AOP; and support for attending meetings and/or travel from MSD, United Therapeutics Corporation, Ferrer, Janssen, and AOP. Cirulis reports grants or contracts from Entelligence (now ATS); and leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid, with ATS Web Committee Co‐Director and ATS Program Committee. Gerges reports grants or contracts from AOP Health; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from AOP Health, Actelion/Janssen, and Ferrer; and support for attending meetings and/or travel from AOP Health, Actelion/Janssen, Ferrer, Daiichi Sankyo, and MSD. Gillmeyer reports grants or contracts from Parker B. Francis Fellowship Award, Doris Duke Charitable Foundation Award, and National Center for Advancing Translational Sciences, National Institutes of Health (BU‐CTSI Grant Number 1UL1TR001430); and leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid, for the American Thoracic Society Program Committee (unpaid). Hobohm reports consulting fees for Janssen, Boston Consulting, and MSD. Lau reports grants or contracts from Janssen and GSK; consulting fees for Janssen; and payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Janssen. Morland reports grants or contracts from United Therapeutics Corporation; leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid, from the PVRI Innovative Drug Development Initiative (unpaid); and stock or stock options from United Therapeutics Corporation. Nikkho reports grants or contracts from Bayer AG; and leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid, from the PVRI Innovative Drug Development Initiative (unpaid). The remaining authors declare no conflict of interest., (© 2023 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.)

Introduction: Mexico ranks second in the global prevalence of obesity in the adult population, which increases the probability of developing dyslipidemia. Dyslipidemia is closely related to cardiovascular diseases, which are the leading cause of death in the country. Therefore, developing tools that facilitate the prediction of dyslipidemias is essential for prevention and early treatment., Methods: In this study, we utilized a dataset from a Mexico City cohort consisting of 2,621 participants, men and women aged between 20 and 50 years, with and without some type of dyslipidemia. Our primary objective was to identify potential factors associated with different types of dyslipidemia in both men and women. Machine learning algorithms were employed to achieve this goal. To facilitate feature selection, we applied the Variable Importance Measures (VIM) of Random Forest (RF), XGBoost, and Gradient Boosting Machine (GBM). Additionally, to address class imbalance, we employed Synthetic Minority Over-sampling Technique (SMOTE) for dataset resampling. The dataset encompassed anthropometric measurements, biochemical tests, dietary intake, family health history, and other health parameters, including smoking habits, alcohol consumption, quality of sleep, and physical activity., Results: Our results revealed that the VIM algorithm of RF yielded the most optimal subset of attributes, closely followed by GBM, achieving a balanced accuracy of up to 80%. The selection of the best subset of attributes was based on the comparative performance of classifiers, evaluated through balanced accuracy, sensitivity, and specificity metrics., Discussion: The top five features contributing to an increased risk of various types of dyslipidemia were identified through the machine learning technique. These features include body mass index, elevated uric acid levels, age, sleep disorders, and anxiety. The findings of this study shed light on significant factors that play a role in dyslipidemia development, aiding in the early identification, prevention, and treatment of this condition., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Gutiérrez-Esparza, Pulido, Martínez-García, Ramírez-delReal, Groves-Miralrio, Márquez-Murillo, Amezcua-Guerra, Vargas-Alarcón and Hernández-Lemus.)

Portal hypertension may have major consequences on the pulmonary vasculature due to the complex pathophysiological interactions between the liver and lungs. Portopulmonary hypertension (PoPH), a subset of group 1 pulmonary hypertension (PH), is a serious pulmonary vascular disease secondary to portal hypertension, and is the fourth most common subtype of pulmonary arterial hypertension. It is most commonly observed in cirrhotic patients; however, patients with noncirrhotic portal hypertension can also develop it. On suspicion of PoPH, the initial evaluation is by a transthoracic echocardiogram in which, if elevated pulmonary pressures are shown, patients should undergo right heart catheterization to confirm the diagnosis. The prognosis is extremely poor in untreated patients; therefore, management includes pulmonary arterial hypertension therapies with the aim of improving pulmonary hemodynamics and moving patients to orthotopic liver transplantation (OLT). In this article, we review in detail the epidemiology, pathophysiology, process for diagnosis, and most current treatments including OLT and prognosis in patients with PoPH. In addition, we present a diagnostic algorithm that includes the current criteria to properly select patients with PoPH who are candidates for OLT., Competing Interests: The authors declare no funding or conflicts of interest., (Copyright © 2023 The Author(s). Transplantation Direct. Published by Wolters Kluwer Health, Inc.)

Traditionally, monitoring approaches to survey marine caves have been constrained by equipment limitations and strict safety protocols. Nowadays, the rise of new approaches opens new possibilities to describe these peculiar ecosystems. The current study aimed to explore the potential of Structure from Motion (SfM) photogrammetry to assess the abundance and spatial distribution of the sessile benthic assemblages inside a semi-submerged marine cave. Additionally, since impacts of recent date mussel Lithophaga lithophaga illegal fishing were recorded, a special emphasis was paid to its distribution and densities. The results of SfM were compared with a more "traditional approach", by simulating photo-quadrats deployments over the produced orthomosaics. A total of 22 sessile taxa were identified, with Porifera representing the dominant taxa within the cave, and L. lithophaga presenting a density of 88.3 holes/m 2 . SfM and photo-quadrats obtained comparable results regarding species richness, percentage cover of identified taxa and most of the seascape metrics, while, in terms of taxa density estimations, photo-quadrats highly overestimated their values. SfM resulted in a suitable non-invasive technique to record marine cave assemblages. Seascape indexes proved to be a comprehensive way to describe the spatial pattern of distribution of benthic organisms, establishing a useful baseline to assess future community shifts., (© 2023. The Author(s).)

Objective: The objectives of this study were to evaluate an automated device for ventilatory support based on AMBU manufactured in March 2020., Methods: The ESSI-1 INC was evaluated through pulmonary mechanics and physiology parameters through compensatory spirometer tests (TISSOTs), and an artificial lung Model5600i Dual Adult PNEU VIEW SYSTEM; it was also compared to the anesthetic ventilatory support equipment (AEONMED 7500) in porcine models, measuring ventilatory, hemodynamic and gasometric parameters., Results: This equipment (ESSI-1 INC) was successfully tested by mechanical and biological models, such as pigs in which its performance was evaluated in terms of variability of tidal volume, ventilation frequency, and I/E relationship versus the manual performance of two medical interns. All the results turned out as expected and were satisfactory., Conclusions: It is safe and effective equipment and should be tested and used in diverse clinical conditions to standardize the ventilatory safety and care of patients who require it., (Copyright: © 2023 Permanyer.)

Introduction: In SERAPHIN, a long-term, event-driven, double-blind randomised controlled trial in pulmonary arterial hypertension (PAH), macitentan 10 mg significantly reduced the risk of morbidity/mortality compared with placebo. Its open-label extension study (SERAPHIN OL) further assessed long-term safety and tolerability of macitentan 10 mg in PAH patients., Methods: Patients in SERAPHIN who completed the double-blind treatment period or experienced a morbidity event during the study could enter SERAPHIN OL. Patients received macitentan 10 mg once daily, and safety and survival were assessed until end of treatment (+ 28 days). Two overlapping sets were analysed for safety: (1) all patients in SERAPHIN OL (OL safety set); (2) patients randomised to macitentan 10 mg in SERAPHIN (long-term safety/survival set). Survival was evaluated as an exploratory endpoint in the latter set., Results: Of 742 patients randomised in SERAPHIN, 550 (74.1%) entered SERAPHIN OL (OL safety set); 242 patients were randomised to macitentan 10 mg in SERAPHIN (long-term safety/survival set). Median (min, max) exposure to macitentan 10 mg was 40.1 (0.1, 130.5) months (2074.7 patient-years; OL safety set) and 54.7 (0.1, 141.3) months (1151.0 patient-years; long-term safety/survival set). Safety in both analysis sets was comparable to the known safety profile of macitentan. Kaplan-Meier survival estimates (95% CI) at 1, 5, 7 and 9 years were 95.0% (91.3, 97.1), 73.3% (66.6, 78.9), 62.6% (54.6, 69.6) and 52.7% (43.6, 61.0), respectively (long-term safety/survival set; median follow-up: 5.9 years)., Conclusions: This analysis provides the longest follow-up for safety and survival published to date for any PAH therapy. The safety profile of macitentan 10 mg over this extensive treatment period was in line with that observed in SERAPHIN. As the majority of patients were receiving other PAH therapy at macitentan initiation, our study provides additional insight into the long-term safety of macitentan, including as part of combination therapy., Trial Registration: ClinicalTrials.gov Identifiers: NCT00660179 and NCT00667823., (© 2022. The Author(s).)

Riociguat, a soluble guanylate cyclase stimulator, is approved for treatment of adults with pulmonary arterial hypertension (PAH). The safety, tolerability, and pharmacokinetics (PK) of oral riociguat in a pediatric population with PAH was assessed in PATENT-CHILD (NCT02562235), a multicenter, single-arm, 24-week, open-label, Phase 3 study. Patients aged 6-17 years in World Health Organization functional class (WHO-FC) I-III treated with stable endothelin receptor antagonists and/or prostacyclin analogs received riociguat equivalent to 0.5-2.5 mg three times daily in adults, as either oral pediatric suspension or tablets, based on bodyweight. Primary outcomes were safety, tolerability, and PK of riociguat. Twenty-four patients (mean age 12.8 years), 18 of whom were in WHO-FC II, were enrolled. Adverse events (AEs), mostly mild or moderate, were reported in 20 patients (83%). Four patients (17%) experienced a serious AE; all resolved by study end and two (8%) were considered study-drug related. Hypotension was reported in three patients and hemoptysis in one (all mild/moderate intensity). Riociguat plasma concentrations in pediatric patients were consistent with those published in adult patients. From baseline to Week 24, mean ± standard deviation increase in 6-minute walking distance was 23 ± 69 m ( n  = 19), and mean decrease in NT-proBNP was -66 ± 585 pg/ml ( n  = 14). There was no change in WHO-FC. Two patients experienced clinical worsening events of hospitalization for right heart failure. PK results confirmed a suitable riociguat dosing strategy for pediatric patients with PAH. The data suggest an acceptable safety profile with potential efficacy signals., Competing Interests: H. G. A. reports fees for lectures and member of advisory boards for Bayer and Janssen. D. D. I. reports the University of Colorado contracts with Acceleron, Actelion/Janssen, Altavant, Bayer, Eli Lilly, GlaxoSmithKline, and United Therapeutics for D. D. I. to be a consultant and perform clinical trials. S. M. reports consultancy from Actelion/Janssen and GlaxoSmithKline. J. P. reports fees for lectures and/or consultancy from Actelion/Janssen. T. P. reports fees for lectures, member of advisory boards, and research grants from Actelion/Janssen, Bayer, GlaxoSmithKline, and Pfizer. H. Y. reports research grants and lecture fees from Actelion, AbbVie, and Teijin Pharma; research grants from Tanabe‐Mitsubishi; lecture fees from Bayer, GE Healthcare Japan, Merck Sharp and Dohme, Novo Nordisk, Nihon Seiyaku, Nihon Shinyaku, Pfizer, and Termo. G. C., M. G., S. L., A. R., S. S., S. W., G. W. are employees and stockholders of Bayer AG. D. B. is a steering committee member for Eli Lilly and Company, and steering committee and advisory board member for Actelion Pharmaceuticals, Bayer, and Novartis. M. B. reports fees for lectures and/or consultancy from Acceleron, Actelion/Janssen, AOP, Bayer, Eli Lilly, GlaxoSmithKline, and Gossamer; unrestricted research grants from Actelion and Bayer. The remaining authors declare no conflict of interest., (© 2022 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.)

Savalia savaglia is an ecosystem engineer listed as Near-Threatened by the IUCN, even though effective management and proper monitoring efforts to assess its distribution is still lacking. The record of large, long-established colonies can indicate the occurrence of areas with limited human local pressure. These areas may be considered as proxies for the creation of baselines of reference useful to design restoration strategies. The aim of this work was to update the distribution of S. savaglia Mediterranean populations to develop an Ecological Niche Model, highlighting potential areas for future monitoring programs. Occurrence data were collected and harmonized into a single dataset using the scientific literature and validated observations to feed a presence-only MaxEnt model, obtaining a basin-level potential distribution of the species. The results of our study can support decision-makers in marine spatial planning measures including the preservation of mesophotic environments and prioritizing areas for conservation., (Copyright © 2022. Published by Elsevier Ltd.)

Pulmonary arterial hypertension (PAH) is a severe clinical condition that significantly affects patients' quality of life and survival. Since the emergence of prostanoids 45 years ago, different drugs acting on vasoconstriction/vasodilation mechanisms have been developed for the treatment of PAH. Current evidence shows that better results occur when combined therapy is initiated up-front with periodic and systematized evaluations for escalation and switching. Among these strategies, riociguat has a relevant role, supported by the results of several clinical studies. This document issues recommendations by a panel of experts who analysed and discussed the indications and limitations for riociguat in PAH in different institutions of the Mexican health system., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Treatment for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension in Latin America differs between countries, with regard to disease etiology, health insurance coverage, and drug availability. A group of experts from Latin America, met to share regional experiences and propose possible lines of collaboration. The available evidence, regional clinical practice data, and the global context of the proceedings of the 6th World Symposium on Pulmonary Hypertension, held in Nice, France, in February 2018, were analyzed. Here, we discuss some priority concepts identified that could guide transnational interaction and research strategies in Latin America: (1) despite being evidence-based, the 6th World Symposium on Pulmonary Hypertension proceedings may not be applicable in Latin American countries; (2) proactive identification and diagnosis of patients in Latin America is needed; (3) education of physicians and standardization of appropriate treatment for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension is vital; (4) our clinical experience for the treatment strategy for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension is based on drug availability in Argentina, Brazil, Colombia and México; (5) there are difficulties inherent to the consultation of patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension, and access to treatment; (6) the importance of data generation and research of Latin American-specific issues related to pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension is highlighted., (© 2021 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.)