30 June 2022

Meeting report: PVRI2022 Athens

PVRI2022 Athens took place from Wednesday 22 June – Sunday 26 June 2022 and was attended by over 350 delegates from around the world.

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Organisers Lan Zhao (left) and Bradley Maron (middle) present awards with CEO Stephanie Barwick (right) at the Gala Dinner

Wednesday featured an agenda packed with meetings from our Task Forces. The 3rd Infection in Pulmonary Vascular Disease (iPVD) Symposium began with a keynote lecture by Catherine Blish, who has led national and global efforts to understand the contribution of inflammation in the development of COVID-19-related pulmonary disease. Through the use of advanced Omics techniques and patient samples, she has identified a unique immune signature that correlates with the disease severity and provides insight into possible therapeutic avenues. Brian Graham presented epidemiological data on Schistosoma PH incidence and prevalence in the world and updated us on his efforts to collaborate with groups in Africa and Latin America to build patient registries that will capture the clinical evolution of these patients and lead to improvement in public health policies aimed at preventing infection. Other highlights from the symposium included lectures by Kurt Stenmark and Francisco Perez Vizcaino addressed the role of the complement system and ACE2 in the pathogenesis of COVID-19-related lung disease. Provocative presentations by Navneet Dhillon and Mathias Clauss addressed the role that circulating vesicles play in regulating how HIV and COVID-19 interact with the pulmonary circulation to regulate short and long-term responses to infection. Other provocative discussions led by Peter Nyasulu and Nicola Petrosillo centred on the impact that co-infection with HIV or Schistosoma has on PH patient morbidity and mortality in Africa and Italy and how COVID-19 infection influences the clinical course in this context. The symposium finished with presentations by Luke Howard, Rudolf Oliveira, and Andrew Bryant addressing how long-term COVID complications may influence exercise tolerance, right ventricular function, and lung mechanics in PH patients.

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Ghazwan Butrous, Sandra Medrano-Garcia, Vinicio de Jesus Perez and Angel Cogolludo attend the iPVD meeting

During the Imaging Task Force meeting the group set ambitious targets for using imaging, AI and advanced statistical approaches to improve risk stratification in PAH. Differences were discussed in imaging across the globe and proposals to work on a statement to tailor diagnostic strategies to imaging availability.

David Kiely gave a “Big thanks to Stephanie Barwick for inspiring the Imaging Task Force and reminding us that purpose, passion and people are what make meetings like today such a success. A special mention to Lucas Celant for a great talk and support from team Amsterdam”

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A group photo from the Imaging Task Force meeting

The PVRI Paediatric Task Force Group met to discuss several key topics related to the role and utility of classification systems and registries in generating real world data for enhancing care, research and improving clinical trial design.

Maria Jesus del Cerro discussed issues that led to the development of original PVRI Paediatric Classification System in Panama over 11 years ago, including its impact on understanding the complexities of providing more precise clinical phenotyping of paediatric PH. This system first identified the multifactorial nature of childhood PH and included important recognition of distinctions of diseases and outcomes that were unique to paediatric issues that could augment the use of the more traditional WSPH Classification System.

Plans were discussed to formulate a working group to explore incorporation of advanced genetics and additional biomarkers with clinical features to optimize its application in paediatrics. Several research and cases were presented for discussion among the entire group, including novel echocardiographic metrics to better identify at risk children. Finally, the formation of the Paediatric Clinical Trials and Endpoints subcommittee within the PVRI-IDDI programme were discussed along with plans to develop working groups to address key themes that would include teams from the clinics, pharmaceuticals, regulatory and patient advocacy groups to focus on key topics directed to brining novel therapies to clinical care in diverse paediatric PH diseases.

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A group photo from the Paediatric Task Force Group meeting

The Position-General consensus of the Exercise Task Force meeting led by David Systrom, Aaron Waxman and Rischard, was that upright exercise is preferable due to the typical natural state of exercise. There was consensus within the group that a study examining the hemodynamic differences in position on the same patient (with two exercise bouts) be done. The respiratory variation discussion centered around the use of respiratory variation in differentiation of WSPH G1 versus G2 PH and the measurement of PCWP. There was consensus that respiratory averaging is preferred based on hemodynamic assessment with esophageal balloons particularly based on data in the obese and COPD patients. Later in the meeting, several ideas for a study to examine the accuracy of end-expiratory versus respiratory averaging were discussed. Esophageal balloon placement was generally considered unfeasible across a large group of patients. The slope of a PCWP/CO, absolute value of PCWP obtained at peak exercise, and PCWP after fluid bolus post exercise were all entertained as possible “gold-standard” to examine this question outside of esophageal balloons.

Another gold-standard presented during the meeting was to examine patients within the current HFpEF diagnostic algorithm that have very high or very low risk of HFpEF such that they currently are thought not to need RHC (a practical gold standard). There were safety considerations: the use of fluoroscopy in obtaining PCWP was discussed in this context. Generally, for most patients this is not an issue. For some patients with very high PAP, sometimes adjustment of the catheter is needed. BWH will pull back slightly and gradually reinflate the balloon. UA uses fluoroscopy at low dose (3.5 fps). Occasionally, a wire is needed to insert and keep the catheter in position to advance to wedge with high PAP. With regards to application of exercise testing to specific populations and conditions. The topic of respiratory/ventilatory inefficiency was discussed briefly in the context of clinical meaning and definition. It is especially pertinent in the context of CFS/ME and PASC (post-COVID) syndrome RV-PA coupling with exercise. There was discussion regarding the phenotypes of RV compensation (increasing Ees with exercise) versus decompensation (stable or decreasing Ees with RV dilation) at exercise to maintain. OMICS-metabolomics and proteomics were briefly mentioned in regard to phenotyping specific populations of PH. Phenotyping the use of exercise testing to phenotype PH from a diagnostic, prognostic, and potential clinical trial inclusion (enrichment strategy based on exercise phenotype) was briefly discussed. Pulmonary vascular distensibility-the modifiability of distensibility is unknown in PH especially with current therapies. This is important especially with potential disease-modifying drugs in the pipeline to have an idea of what background therapy does to distensibility.

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Exercise Task Force leaders David Systrom (left) and Aaron Waxman (right) attend a meeting in the main hall

Thursday started with a very strong talk entitled ‘The interaction between drug exposure and genetics as a driver of PAH', by Marc Humbert, followed by Kara Goss who presented ‘The importance of perinatal events to developing PH in early adulthood’. The collaborative spirit of the roundtable was clear, with vibrant interaction on biological and clinical registries during our joint session with the PHA. An important talk was also given by Silvia Ulrich on current state of the art adaptations to high altitude.

Later, a provocative roundtable took place on the relationship “or lack thereof” between COVID and future PH. Mark Gladwin then presented compelling insight on new therapeutic opportunities in patients with PH in HFpEF. Anna Hemnes provided much needed context on the importance of hemodynamics in lung disease prognosis. In his talk, William Oldham gave an elegant presentation challenging the expected facts of hypoxia on metabolism in fibroblast. Thursday ended with an insightful talk on ‘Pollution, global warming and other earthly causes of PAH by this year’s recipient of the Rupert Swift award, Ana Mocumbi.

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Ana Mocumbi delivers the Rupert Swift Award lecture

On Thursday evening we recapped a year in review and waved a special goodbye to our CEO, Stephanie Barwick in the PVRI Annual General Meeting. 

Stephanie gave an overview of the successful PVRI2021/22 webinar series and updated us on progress from pharma, our Regional & Disease Task Forces and the activities of the IDDI. We recapped a year of PVRI grant awards as well as some general updates on PVRI finance. Stephanie finished her talk by summarising PVRI progress and highlights during her tenure as CEO over the past eight years. The talk ended with an emotional standing ovation for Stephanie, followed by a networking reception by the pool.  

“Our Congress in Athens was the most wonderful occasion that marked the ‘end of an era’ for me. I was so touched by the overwhelming outpouring of affection and warmth towards me from everyone. I am truly grateful for all your good wishes and kind sentiments. Your support to me over the past eight years has been invaluable. Thank you for making my time at PVRI so special and giving me such a perfect send-off in Athens. I shall treasure the many fond memories. With my most humble thanks." - Stephanie Barwick, CEO.

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We wave a special farewell to CEO Stephanie Barwick at the PVRI Annual General Meeting

On Friday, Sebastian Bonnet delivered an exceptional talk on new trends, challenges, and great opportunities for publishing scientific work. Later, a truly cutting edge and exciting presentation was given on sub-cellular metabolite imaging in pulmonary vascular cells from Matthew Steinhauser. Beata Wojciak-Stothhard led a riveting discussion on lung-on-a-chip technology updates and how it can be used for understanding PAH better. Audiences learned about state-of-the-art research on the use of remote monitoring for PAH clinical trials from Evelyn Horn as well as the potential for home daily step count as a clinical trial endpoint from Evan Brittain. Later in the session, Paul Yu and David Badesh described the story of Sotatercept: a successful marriage of science and clinical trial work. Edda Spiekerkoetter then discussed the important under studied problem of clinical HHT, while Frédéric Perros and David Montani joined forces to discuss amazing progress in PVOD.

In the afternoon, Anjali Vaidya led a vibrant session on the molecular mechanisms, imaging, and clinical updates in CTEPH. This was anchored by a powerful presentation from Marion Delcroix. A fantastic rapid fire poster session followed that showcased clinical research from Eileen Harder, Elizabeth Bird and Azar Kianzad. This came between moderated poster sessions, which were full of meaningful dialogue and featured the latest exciting research in PH. The organisers gave thanks to Sudarshan Rajagopal for an excellent presentation on molecular imaging in CTEPH which complimented a great presentation from Nick Rahaghi on the approach to imaging analysis. The evening ended with the gala dinner by the pool, where we celebrated some amazing achievements in the PH community.

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A beautiful setting by the pool for the PVRI2022 Athens gala dinner

Saturday, began with a blockbuster discussion on clinical trial updates from a series of world experts including Valerie McLaughlin, Aaron Waxman, and Gerald Simonneau. Manreet Kanwar delivered a compelling and engaging discussion on lessons learned from the VICTORIA trial and persistent challenges in the treatment of left heart disease-pulmonary hypertension.

The pathobiology of right heart failure has advanced, and PVRI had it covered. Ruaa Al-Qazazi shared her new data, hot of the press, on the NLRP-3 macrophage pathway in right ventricular dysfunction. Tim Lahm and Frances de Man dazzled the audience with fantastic insights into genetic risk factors and protective mechanisms that regulate RV function in pulmonary hypertension. Later, Khodr Tello crystalized RV shape, size, and function throughout a stellar presentation that brought to life the concepts of RV-pulmonary arterial coupling and many other right ventricular pathophysiologic principles. Also in the session, Kurt Prins, Patricia Thistlethwaite, and Athenais Boucly taught us all how to make good clinical decisions in complicated situations, including pericardial effusion, pulmonary artery aneurysm, and first-time treatment selection for PAH patients. The organisers thanked Rachel Damico for a fantastic presentation on the role of endostatin in interstitial collagen and endothelial dysfunction - illustrating in new light the importance of matrix remodeling to vascular dysfunction in PAH.

Ryan Tedford and Thenappan Thenappan had an energising and spirited debate about pulmonary artery wedge pressure, timing of RHC, and PAH diagnosis. We also had exercise covered in the final session of PVRI2022, with excellent discussions led by Susanna Mak, Luke Howard, and Gabor Kovacs.

Can exercise be used for precision medicine? Rudolf Oliveira answered just that question with a great presentation on using network medicine, biomarkers, and other strategies to improve CPET interpretation. Another big thanks went to Eloara Ferreira, David Systrom, and David Langleben for their excellent work moderating the exercise session and stimulating a fantastic discussion as the sun was setting in Athens.

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Rudolf Oliveira delivers a talk entitled 'Do exercise pathophenotypes advance personalised medicine in pulmonary vascular disease?'

Sunday as the conference drew to a close, the 3rd annual PAH-ICON symposium was attended by investigators with an interest in the genetics and Omics of PAH from across the world. The many excellent presentations included a summary of the curation of PAH genes and variants by Wendy Chung, and the consensus statement on clinical genetic testing presented by Christina Eichstadt. Both of these will be published as manuscripts soon. Other presentations included plans for the follow up of unaffected mutation carriers to identify triggers for disease and approaches to interrogate the non-coding regions of the genome using machine learning approaches.

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The PAH-ICON group listen to Wendy Chung deliver an 'Update from ClinGen Task Force on PAH genes'

Thank you to the our community for making this year's meeting another great success. We were delighted to see everybody in person once again for an excellent meeting that featured the field's highest quality science. We hope to see you next year - details will be announced shortly.

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A wonderful evening at the welcome reception with drinks, food and some overdue conversations with both old and new colleagues


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