25 January 2018

Task Force Annual Report - Europe

2017 report


The first meeting of the PVRI European Task Force took place at Santa Flavia, in a splendid location near Palermo, Italy, on 6 October 2017.

The host was Dr Patrizio Vitulo, Medical Chief of the Lung Transplant Programme at ISMETT - an Institute for Scientific-based Care and Research (IRCCS) in the fields of end-stage organ failure treatment and research and a Transplant Centre serving as Reference Hospital for the entire Mediterranean area.

The Scientific Committee included Dr Stefano Ghio and Professor Dario Vizza, as co-leaders of the European Task Force, and Dr Patrizio Vitulo.

There was an astonishing turn out with faculty from Europe and USA with approximately 100 delegates attending from 30 Italian centres.

The meeting was introduced by Paul Hassoun, President-elect of PVRI, who reminded everybody of the mission of the PVRI: education and research.

AMIP President Vittorio Vivenzio presented the Association’s mission and activity and described its recent European collaborations. AMIP is one of the two Italian associations established for patients with pulmonary hypertension.

Five round tables focused on the gaps in evidence in the 4 PH groups. On each round table, an introductory presentation summarised the most important clinical issues to be debated, in an open and proactive setting, by the audience and international experts. Most importantly, the time dedicated to the discussion was longer than the time dedicated to the presentation.

In group 1 (PAH) Khodr Tello and the discussants, Sean Gaine and Dario Vizza, debated the best PAWP threshold for differentiation between PAH and post-capillary pulmonary hypertension. They addressed the issue of a possible misclassification of pulmonary hypertension when PAWP is taken as an isolated parameter, ignoring the clinical context and imaging. An interesting discussion took place on the additional value of the fluid challenge test for border-line cases. Furthermore, it was emphasised that PAWP levels were associated with different responses to PAH specific drugs: the lower the PAWP value, better the response.

In the second part of the session, Tomas Pulido discussed the results of the recent morbi-mortality trials showing a significant reduction of the event rate, but a mild effect on classical surrogate endpoints such as 6MWT, BNP and WHO functional class. He concluded that the surrogate endpoints were very useful for individual use but composite morbi-mortality endpoints in event-driven studies provided more important information.

Finally, Raymond Benza discussed the use of risk scores/equations and risk tables. He presented some case studies and emphasised that the ESC/ERS table was more useful to predict a low risk. In contrast, the REVEAL risk score was better to predict an intermediate or high risk.

In Group 2 PH Michele D’Alto introduced the debate presenting the latest evidence on the hemodynamic definitions of PH-LHD.

Recently, such definitions have been modified in the European Guidelines to better approach the characteristics required to reflect the presence of pulmonary vasculopathy. Two different types of post-capillary PH have been identified, ie isolated post-capillary pulmonary hypertension (Ipc-PH) and combined post-capillary and pre-capillary pulmonary hypertension (Cpc-PH). This has fuelled an intense debate in the literature. Another hot topic was whether the pathophysiologic determinants of PH were different in HFrEF vs HFpEF patients, as new data in the literature seemed to suggest. Chair Stefano Ghio and two discussants, Robert Naeije and Stylianos Orphanos, stimulated the discussion by involving the audience and forcing experts to provide their personal ideas and beliefs over and above what the guidelines suggested.

In Group 3 John Wort and Patrizio Vitulo introduced Lucilla Piccari, from August Pi i Sunyer Biomedical Research Institute – IDIBAPS, Barcelona, with the topic on two major gaps in evidence: are the hemodynamic thresholds suggested by the European Guidelines to grade the severity of PH useful to start treatment in Group 3 PH patients? Is it time to differentiate between PH-COPD and PH-IPF?

During the lively discussion, the supporting evidence distinguishing PH-COPD and PH-IPF based on genetics, pathophysiology, and response to specific PH therapies was recounted.

Millions of people over the world suffer from that condition. While promising pilot studies suggest a haemodynamic benefit in severe PH-COPD treated with PH specific drugs, there is major concern for PH-IPF. More questions than answers are still on the table on the definition of the group III phenotype in order to better understand the mechanisms underlying the development of PH in the individual components of Group 3 and the definition of appropriate outcomes for future trials. All the attendees agreed on the need for an international network on Group 3.

In Group 4 the discussion was around the gaps in evidence in CTEPH. Adam Torbicki introduced the topic focusing on the prevalence of chronic thromboembolic disease as a continuum from the persistence of perfusion defect after adequate anticoagulation therapy to the overt disease of CTEPH.

Another point was the definition of persistent PH after a procedure of pulmonary endoarterectomy.

Finally he presented an update on balloon pulmonary angioplasty (BPA) and its indication.

The discussion among the three chairs and the experts in the audience highlighted the need for a better understanding of the pathophysiological link between chronic thromboembolic disease and CTEPH, and how to follow up patients after an episode of pulmonary embolism. Joana Pepke-Zaba discussed the evolving technique of BPA, and how new strategies were able to reduce the occurrence of adverse events. Finally Andrea D’Armini pointed out the need for a second opinion on the patient’s operability before proceeding to PBA.

This ended the meeting and the leaders of the European Task Force invited all to join them again in 2018.

A splendid evening dinner was hosted by the EU Task Force leaders and all delegates enjoyed a wonderful social evening

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