Pulmonary Vascular Research Institute

Annual Report 2016

Pulmonary Vascular Research Institute

PVRI

PVRI Head Office33 St George’s PlaceCanterburyKent CT1 1UTUnited Kingdom

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Pulmonary Vascular Research InstituteAnnual Report 2016

Contents

Pulmonary Vascular Research Institute

PVRI

Report Page

Review from the PVRI President 2016 // Paul Corris 05

Chief Executive Officer’s Report 2016 // Stephanie Barwick 06

Pulmonary Circulation Report 2016 // Jason Yuan & Nick Morrell 10

International Scientific Meetings

10th Annual World Congress in Rome Report 2016 // Sheila Glennis Haworth, Past President 2014-2016 13

3rd Annual Drug Discovery Meeting 2016 // Stuart Rich, John Newman 15

1st Annual Symposium for Young Investigators Overview 2016 // Djuro Kosanovic, Mariola Bednorz

& Michiel Alexander de Raaf 16

Regional Task Force Reports

Central Asia & High Altitude Report 2016 // Max Gassmann & Qadar Pasha 18

China Report 2016 // Zhengo Zhai, Chen Wang & Martin Wilkins 20

Eastern Mediterranean & Saudi Arabia Report 2016 // Paul Hassoun & Majdy Idrees 22

Europe Report 2016 // Stefano Ghio & Dario Vizza 24

India Report 2016 // BKS Sastry 25

North America Report 2016 // Kurt Stenmark & Stephen Archer 26

South America Report 2016 // Gabriel Diaz & Dora Haag 28

South Africa & Sub-Saharan Africa Report 2016 // Paul Williams 30

South East Asia Report 2016 // Helmy Haja Mydin & Roslina Abd Manap 31

Disease & Speciality Task Forces

Exercise Report 2016 // David Systrom & Aaron Waxman 33

Imaging Report 2016 // David Kiely & Andy Swift 34

Paediatric & Congenital Heart Disease Report 2016 // Maria Jesus del Cerro, Ian Adatia & Steven Abman 35

Pre-Clinical & Molecular Science Report 2016 // Mandy MacLean 36

Schistosomiasis Report 2016 // Brian Graham 37

Women’s Health & Pregnancy Report 2016 // Anna Hemnes & Barbara Cockrill 38

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IntroductionIn 2016 the PVRI built on the solid foundations established in 2015 following our governance review. We made good progressas THE professional, global organisation focused on improving the care of patients with pulmonary vascular disease andfacilitating research into this devastating disease. We have, I think, achieved a great deal.

Any charitable organisation requires a sound financial footingand, this year, we were able to consolidate our income both viamore efficient collection of membership dues, increased“Roundtable” core funding from pharma and, of course, amaintained grant from the Cardiovascular Medical Researchand Education Fund. I would like to convey my personal thanksto these latter benefactors. Grateful thanks also to LynetteSwift, who organised a most successful auction and dinner onbehalf of the PVRI in May 2016. As a result of all these efforts,I am happy to advise all our members that we have never beenso financially secure since our inception! Our financial backinghas also been made more secure through specific grantsfrom GSK to fund our e-learning programme, by UnitedTherapeutics to enable us to sponsor PVRI membership forover 60 doctors from the developing world and therecruitment of a new and experienced Treasurer to our Boardand Executive Committee, John Harrington.

I have encouraged our Task Forces to recognise that they tooact as our engines for action and also for them to identify areasof focus on an annual basis, including the sharing of goodpractice. We have established two new Regional Task Forces inEurope and South East Asia and a new joint Pharma Topic TaskForce whose remit is to help facilitate joint academic interestsand aid collaboration. There are new faces at the helm of someof our existing Task Forces to help energise fresh activities.

Another exciting piece of news is the signing of a Memorandumof Understanding between The Dinosaur Trust and the PVRI.The Dinosaur Trust was set up by Jamie Strachan and his wifeto support research into PAH via the organisation of charitableevents, this has been very successful. PVRI will work alongsideThe Dinosaur Trust to provide a Scientific Committee toestablish a mechanism for grant applications and theirassessment. This is an important and strategic alliance for bothparties.

In addition to our very successful Annual World Congress inRome and the Drug Discovery & Development Symposium inBethesda, there were numerous PVRI symposia at majormeetings, including the ISHLT, the Malaysian Thoracic Societyand the Cuban Cardiac Society (scheduled in 2017) togetherwith stand-alone PVRI associated meetings, including the High-Altitude meeting in Central Asia, the Eastern Mediterranean(SAPH) meeting and Indian meetings.

In 2016 we also held the inaugural Committee for YoungClinicians & Scientists Symposium organised by, and runspecifically for, the younger members of the PVRI.

The PVRI’s journal Pulmonary Circulation has experienced majorbeneficial changes in 2016 whilst maintaining its high standardthanks to the drive and initiative of its Editors, Jason Yuan andNick Morrell, ably assisted by Michael Brown and MaryReynolds. The journal is now all set for the next decade with achange in publisher leading to significant advantages in themedium term for the PVRI and the expectation of a satisfactoryinitial Impact Factor in 2017.

We are only now beginning to exploit the huge potential of thePVRI as a global organisation committed to helping patientswith pulmonary vascular disease. There is much work to do butwith your enthusiastic membership and a cohort of brightyoung colleagues we can expect to go far.

I hope you agree with me that 2016 has been a big step in thelong walk we face. Importantly, the PVRI remains true to itsaims and ensures a definitive focus on helping to improve thecare of patients with PH living in the developing world.

Finally, in my current role as President of the PVRI, I would liketo thank our CEO Stephanie Barwick for all her hard work andunfailing commitment to the PVRI. She is ably supported by herteam in the UK, Aaron Shefras, our media guru; AdministrativeAssistant Margaret Carver who will continue in a part-time roleand our newcomer Georgie Sutton; as well as colleagues in theUSA, Andrea Rich, our Events Manager, and Mary Reynolds,PC Editorial Associate. Their contributions have been essentialto the advances made in 2016.

The day to day course of the PVRI is steered by the ExecutiveCommittee, advised by its Council, and their collective wisdomhas been a great source of strength during 2016.

I am personally humbled and grateful for the confidence shownin electing me to be your President, a position I am immenselyproud of, and assure you that I intend tocontinue to work tirelessly on yourbehalf to maintain progress andsuccess in 2017.

Review from the PVRI President2016

Professor Paul A Corris MD

President PVRI

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Annual Report 2016

PVRI Chief Executive Officer’s Report 2016Stephanie Barwick, CEO

2016 has been a year of steady growth, consolidating our strengths as the only global independent voice of authority inthe field of pulmonary vascular disease. The outline below highlights some of our achievements and operational activitiesduring the year.

OPERATIONAL ACTIVITIES

PVRI Board of Directors/Trustees Meetings & AnnualGeneral MeetingBoard of Director meetings, to oversee the strategic directionof the PVRI, were held twice in 2016 - in January in Rome andin July in Bethesda, ahead of our two major scientific meetings.The Annual General Meeting, uniting all our members, washeld on the first evening of our 10th Annual World Congressin Rome on 14 January 2016.

Changes to the Board included the handover of thepresidency from Sheila Glennis Haworth to Paul Corris andthe appointment of Paul Hassoun as President Elect. Aftermany years of service, Sir Simon Campbell, Treasurer of thePVRI, resigned from the Board in January and in July wewelcomed John Harrington to the Board and ExecutiveCommittee as the PVRI’s new Treasurer. We would like toexpress our sincere thanks and best wishes to Simon for all hishard work and support to the PVRI since its inception in 2007.A big welcome to John and good luck in his new role.

PVRI FinancesThanks to the continued Roundtable membershipcontributions from the pharma industry, support from theCardiovascular Medical Research and Education Fund(CMREF), income from registration fees to our scientificmeetings and membership contributions, effective fundraisingactivities and income received for special projects; we are nowin a solid position to take the PVRI to the next level. Accuratefinancial monitoring of income and expenditure has resultedin regular management accounts for review by the ExecutiveCommittee and annual audited accounts. During the lastannual audit of the PVRI 2015 UK accounts, we have receivedthe highest rating with no recommendations for improvement.Well done to everyone involved!

New Members of StaffDuring the year, our two different offices in the UK and USAwelcomed new members of staff. In the USA, following theresignation of Annisa Westcott, we recruited Mary Reynoldsin June as ‘Editorial Associate’ for our journalPulmonary Circulation. She is based in Arizonawith PC’s Chief Editor Jason Yuan, workingalongside our Publishing Consultant MichaelBrown.

In the UK, we recruited Georgie Sutton to theteam in November as full-time Marketing &Admin Officer to provide consistent adminsupport to the office and to support AaronShefras, PVRI Marketing Manager, with someof our activities.

A big welcome to Georgie and Mary and thanks to both for allyour contributions so far.

MARKETING ACTIVITIES

New Joint PVRI/PC Website and Social MediaOur new website was officially launched on 14 January 2016during the AGM. This investment certainly paid off with a310% year on year increase on site views. A total of 245,311page views was recorded during 2016, compared to 80,437in 2015. The homepage film has been viewed over 13,000times and nearly 60% of visitors engaged with our site, visitingmore than three pages. We now have over 870 e-learningmaterials on the website which consist of lecture recordings,interviews, atlas images, abstract submissions and PC journalarticles, all linked together via search words and relatedcontent criteria. Our e-learning materials have been viewedby 43% of visitors who also click on four or more pages ofrelated content.

Facebook likes have increased by 45% to 805 and Twitterfollowers are up by 11% to 533. Electronic adverts promotingthe PVRI and our Annual World Congress in Miami wereplaced in PH News reaching out to over 6,000 PHprofessionals in the USA. Also, there were email campaignsby the International Society for Heart & Lung Transplantation(ISHLT), the Pulmonary Hypertension Association (PHA) inthe USA and the American Heart Association (AHA), to alltheir members. Thank you to everyone who has helpedpromote our Congress.

The PVRI online newsletter featuring the ‘President’s blog’and other current topics was produced quarterly, reaching outto over 6,000 PH professionals in 75 different countriesfrom across the world.

This is a great result and a massive thank you goes to AaronShefras, our Marketing Manager.

INTERNATIONAL SCIENTIFICMEETINGS

As has become tradition in the PVRI calendar, during 2016 weheld two major international conferences - the 10th AnnualWorld Congress on PVD in Rome, Italy, from 14-19 Januaryand the 3rd Annual Drug Discovery & DevelopmentSymposium in Bethesda, USA, on 11-12 July. In addition tothese, the PVRI Committee for Young Clinicians & Scientists(CYCS) organised its 1st Annual Symposium for YoungInvestigators, which was held in Germany from 8-12September.

Annual World Congress After many years, we decided to organise our 10th PVRIAnnual World Congress on PVD in Rome without a partnerorganisation - a big risk that paid off: 223 internationaldelegates from 28 different countries enjoyed the active

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Annual Report 2016

discussions over the three and a half days of the Congress. 73%of delegates rated the overall scientific programme as ‘excellent’.The most popular topics were ‘Advances in our understanding ofPVD’ and the session on ‘Chronic Thromboembolic PH’. Manythanks to our scientific meeting planners Dario Vizza and StefanoGhio for putting together a great programme and to Andrea Rich,our Events Manager, for the exciting mix of social activities,including Vatican tours and pasta cooking courses. During theGala Dinner on Saturday evening, we presented the PVRIAwards.

2015 PVRI Award WinnersDespite a lengthy bus journey to the Gala Dinner restaurantthrough the hidden streets of Rome, we finally celebrated theachievements of some of our most distinguished and activemembers and President Paul Corris presented the followingawards:

• The Lifetime Achievement Award 2015was presented toLewis Rubin in recognition of his distinguished contributionto patient care and research in PVD.

• The Achievement Award 2015went to Nick Morrell, ChiefEditor of Pulmonary Circulation, for his outstandingcontribution to medical research, the PVRI and its journalPulmonary Circulation.

• The Certificate of Excellence 2015was presented to Ian Adatia and thePaediatric & Congenital Heart DiseaseTask Force in recognition of theiroutstanding contributions to the PVRI.

Congratulations to all our winners.The prize winners of 2016 will beannounced at our Gala Dinner in Miamiin January 2017.

Drug Discovery & DevelopmentSymposiumOur Bethesda Drug Symposium wasattended by 112 delegates from academia,industry and regulatory bodies whoparticipated in stimulating discussions anddebates over a day and a half. Sincerethanks to the three Co-chairs Stuart Rich,John Newman and Surpil Erzurum for

putting together a diverse scientific programme featuringexcellent talks on potential new targets for therapy, includingBMPR2, estrogen and mitochondria. There was also a greatdemonstration that exercise induced PH is a real clinical entityleading to symptomatic breathlessness, a topic which receivedmuch interest from the audience.

Symposium for Young InvestigatorsCYCS Chair Djuro Kosanovic together with Mariola Bednorzorganised the inaugural 1st PVRI Annual Symposium for YoungInvestigators, which was held in the beautiful Castle Waldeck inGermany in September. Twelve young scientists from around theworld attended the event which was spread over a weekend andincluded stimulating scientific sessions as well as an exciting socialprogramme enjoyed by all delegates. Our thanks to Djuro andMariola for organising a great event. We hope that this willbecome a regular annual meeting attracting more youngresearchers from around the world.

Last, but not least, a big thank you to Andrea Rich, the PVRIEvents Manager, for looking after all the logistics of our scientificmeetings and making sure everything runs smoothly. This is by nomeans a small task. Thank you Andrea!

For more information on all our international scientificmeetings, please have a look at pages 13-16.

MEMBERSHIP

PVRI MembershipThe PVRI network has expanded to over 6,000 people spreadacross 75 different countries worldwide, including an activemembership of 1,250 PH professionals. We are immensely proudof our international reach and global representation as the PVRIis the only global charity dealing in PVD. Thank you to everyonefor your continued support.

Task Force ActivityThe unsung heroes of the PVRI are most definitely our Task Forceleaders and active members throughout the world. It is throughthem that we fulfil our mission and bring the PVRI to the rest ofthe world. A sincere thanks to everyone who is involved in a Task

Force, leaders and members, for all their hardwork, commitment and support.

Our Task Forces have had an active year withmany regional PH meetings held in Jordan,Bolivia, India, USA, Europe and China. During2016 we formed a new Regional Task Force inSouth East Asia with Task Force leaders HelmyHaja Mydin and Roslina Abd Manap stimulatingPH activity in Malaysia and Singapore and theleadership of the South Africa & Sub SaharanAfrica Task Force has been passed to PaulWilliams from Karen Sliwa-Hahnle and AnaMocumbi. Our thanks go to all present and pastTask Force leaders for their immense hard workand a warm welcome to everyone who hasjoined during the year.

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Annual Report 2016

The newly established Pre-clinical & Molecular Science TaskForce, led by Mandy McLean, had its first inaugural meetingahead of the Annual World Congress in Rome withparticipation from 40 scientists, as did the Imaging TaskForce led by David Kiely. A new Pharma Task Force has beenbrainstormed during our Bethesda Drug Discovery &Development Symposium and will have its first officialmeeting during our Congress in Miami in January 2017.

For more information on our Task Forces, please have a lookat pages 18-38.

Industry Support & Roundtable Membership 2016I would like to express my most heartfelt thanks to all ourindustry partners who have supported us during 2016 andengaged in the PVRI Roundtable membership, which is thePVRI-industry alliance. Without their continued sponsorshipand support we simply could not fulfil our mission. Roundtablemembers for 2016 included Bayer, Bellerophon, Gilead, GSKand United Therapeutics.

In addition to the Roundtable membership, particular thanksgo to United Therapeutics and GSK. United Therapeutics hasagreed to sponsor PVRI membership for 67 doctors fromdeveloping countries over two years, which will furtherenhance our mission and focus in the developing world. GSKhas provided substantial financial support over two years forour e-learning initiative. Thank you to both!

Discussions have already started for 2017 and I am delightedto announce that in addition to the 2016 Roundtablemembers, Merck has also agreed to participate anddiscussions have started with Actelion.

PVRI PUBLICATIONS & E-LEARNINGINITIATIVE

Pulmonary Circulation2016 has been an exciting and successful year for our journalwith historic high numbers of citations and downloads ofjournal articles and new members joining the team, includingMichael Brown in January and Mary Reynolds in June.Michael is an experienced Publishing Consultant andcurrently combining the roles of Business Manager andManaging Editor, ably assisted by Mary Reynolds, employedas Editorial Associate to support the Chief Editors JasonYuan and Nick Morrell.

During the year we published a total of 91 articles as well asan additional journal supplement in March, sponsored byBayer, which featured research articles on Bayer’s new drugRiociguat. We are grateful to Bayer for their support.

Kurt Stenmark has been appointed as Depty Editor andwe have formed a Publications Committee chaired by Past-President Sheila Glennis Haworth who oversees thestrategic business plan of the journal, monitoring milestoneachievements.

In addition to the above, we have started the transition of thejournal from University Chicago Press (UCP) to a newpublisher, SAGE, who will take over in January 2017. Ourthanks to UCP for publishing Pulmonary Circulation over thepast three years and helping us consolidate our position inthe market. We look forward to the announcement of ourImpact Factor in the summer of 2017 and the exciting timesthat lie ahead.

We are immensely proud of Pulmonary Circulation and ourthanks go to everyone involved in the journal, but mostimportantly, the Chief Editors Jason Yuan and Nick Morrellwho have made the journal what it is today by devotingendless hours of their voluntary time. We cannot thank themenough for all that they do!

A full report on Pulmonary Circulation is on pages 10-11.

PVRI ChronicleTwo editions of the PVRI Chronicle have been publishedonline during the year in February and October. Many thanksto the PVRI CYCS (Committee for Young Clinicians &Scientists) and Aaron Shefras for all their hard work in puttingthese together.

E-learning CourseFollowing a successful feasibility report in 2015, we have nowstarted to implement its findings and are in the process ofproducing an e-learning course on PVD aimed at pulmonaryvascular clinicians throughout the world. GSK has provided uswith a generous grant over two years to support this initiativeand we have appointed Martin Johnson and Colin Church inGlasgow, UK, to produce the content for the course. We areaiming for this to be a completely unique course in the field ofPH, focusing on various real-life patient case studies, whichwill provide the clinician with an experiential learning toolfor the accurate diagnosis and management of PVD. Anexperienced media agency has been appointed to assist in thedevelopment and we are hoping to launch the first part of thecourse at our Drug Discovery & Development Symposiumin July 2017.

PVRI GRANTS

During 2016 the PVRI supported its membership by awardingresearch and travel grants for speakers to attend variousscientific meetings across the globe.

Travel GrantsNineteen travel grants were awarded during the yearto various PVRI members in support of national andinternational PVRI meetings and initiatives. These includedthe 10th Annual World Congress on PVD in Rome, Italy inJanuary, the SAPH 2016 Annual Symposium in Jordan inFebruary, the La Paz meeting in Bolivia in February and theHigh-Altitude meeting in Issyk-Kul, Kyrgyzstan in September.

Travel Grants for the PVRI CYCSTen travel grants were awarded to the most active membersof the CYCS during 2015 to enable them to attend ourAnnual World Congress in Rome. All winners were presentedwith a certificate during the Gala Dinner.

PVRI FUNDRAISING ACTIVITIES

Annual Fundraising EventUnder the leadership of Lynette Swift, a

committed volunteer directly affectedby PVD through her son, the PVRI heldits second major fundraising event, an

Auction & Dinner on 19 April 2016 at theFoundling Museum in London, UK.

The event was a huge success with over 100 invited guestsattending the dinner and many more bidding for over 60online auction prizes, which included activity and sports days,pieces of art and memorabilia as well as exciting holidaydestinations. A total of £20,000 ($25,000) was raised in netprofits for the PVRI and we are extremely grateful to Lynettefor all her hard work and support.

Agreement with The Dinosaur TrustDuring the year, the PVRI has signed a Memorandumof Understanding with The Dinosaur Trust, a UK Charityfounded in 2013 by Jamie and Julia Strachan, who have ayoung son with PVD. The Dinosaur Trust is extremelysuccessful in raising funds to help those affected by PVD.Both, The Dinosaur Trust and the PVRI, are aligned in theirmissions and this newly established partnership will helpprovide vital funding for some of our most active researchersand clinicians. We look forward to working with The DinosaurTrust in 2017.

I would like to thank everyone for their support during 2016,especially our President Paul Corris for hishard work during the year and hisinspiring vision for the future.

It is a pleasure to serve you all andI look forward to working with youin 2017.

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Annual Report 2016

Stephanie BarwickChief Executive Officer

Canterbury, UK

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Annual Report 2016

PVRI Pulmonary Circulation Report 2016Jason Yuan & Nick Morrell

The year 2016 has been an exciting one for Pulmonary Circulation! We have seen historic highnumbers of citations and downloads of journal articles; we welcomed new editors and staff tothe team; received confirmation of our impending Impact Factor; and embarked on a new partnership with a leadingpublisher. We also revisited our business plan and publishing model for Pulmonary Circulation and put plans in place toimprove services and benefits for our authors and readers in 2017.

In 2016 we published 91articles, including the latestin our series of high-qualityreview articles from the2015 Grover Conference,and two ConsensusStatements.

Growth in ReadershipThere were 103,469 articledownloads as of 1 November2016 and we expect a totalof 124,000 for the full year,an increase of 46% over2015. This growth in thedissemination of articlesis very encouraging andevidence that the journal isseen as an increasinglysignificant outlet for themost important research inpulmonary vascular disease.

New Impact FactorWe have received confirmation that the Pulmonary Circulation’s firstImpact Factor will be released in 2017. This is excellent news andfurther confirmation that our journal has become established as amajor location for communication in our field.

Expanding Editorial TeamIn October 2016, Deputy Editor Kurt Stenmark joined theeditorial team. He is Director of the CardiovascularPulmonary Research Laboratory at the University ofColorado and is a world-leading cardiovascular andpulmonary scientist. Along with the Editors-in-Chief, Dr Stenmark will invite review articles fromauthors, guide manuscripts through the reviewprocess, and participate in strategic planning for the

journal’s future.

Mary Reynolds joined the team as EditorialAssociate. She brings 20 years writing and editingexperience to the journal.

Michael Brown, a PublishingConsultant, joined the team negotiating

a new publishing contract and guiding the journal’sself-sustaining business model in the first year withSAGE.

Journal Supplement on Riociguat Clinical PharmacologyIn March 2016, we published a special supplement on Riociguat,a novel oral soluble guanylate cyclase stimulator developed forthe treatment of pulmonary arterial hypertension and chronicthromboembolic pulmonary hypertension. The supplement containeda series of articles describing the pharmokinetic and pharmodynamiceffects of Riociguat. We are very grateful to Bayer for their generoussponsorship of the supplement issue.

Change of PublisherAfter three successful years with University of Chicago Press (UCP)as our publisher, our contract came to an end in December 2016. Weexpress thanks to UCP for their contribution to the development ofour journal, and we appreciate their advice and support over the pastthree years.

We have a negotiated a multi-year agreement with our new publisher,SAGE Publishing. SAGE is one of the world’s leading journalpublishers and publishes many journals in science and medicine.Pulmonary Circulation will benefit from increased global visibility,faster publication times and SAGE’s commitment to scholarship andinnovation.

Translation and Language Editing ServicesWe are pleased to provide access to translation and language editingservices for authors to make use of before submitting their article. Fora modest fee, the service can provide translation into English fromChinese, Portuguese and Spanish. For authors whose article is alreadywritten in English and who wish to improve the grammar or language,stylistic editing and formatting services are also available.

Sustainable Publishing ModelDuring 2016 we revised our long-term business plan for PulmonaryCirculation. The journal was launched with grant funding support andwe will now be moving towards a self-sustaining model. This meansthat we need to recover our publishing costs and to do so we need toincrease the article processing charges (APCs).

For two years our APCs have been very low in comparison with otheropen access journals at $1,000 for a research article and lower feesfor other article types. Publishing a journal is a labour-intensiveendeavour and with our journals expansion, it has not been possibleto keep the APC below that of other journals. From 1 January 2017,we will be increasing the APC for research articles to $2,000. This willenable us to work towards a sustainable model and continuepublishing the journal through the long term. We are pleased to saythat the APC still remains considerably lower than the majority ofopen access journals in our field.

Promotional ActivitiesPulmonary Circulation’s Editors promoted the journal at variousinternational conferences, including the PVRI Annual WorldCongress, the American Thoracic Society International Conferenceand the American Heart Association Scientific Sessions. In addition,we have increased promotional email blasts, continue to growour mailing lists and are sustaining our work on developing a socialmedia presence for the journal and increasing its visibility onvarious platforms.

Looking Forward to 2017The changes we have implemented during 2016 will bring a numberof benefits for our authors and readers. We plan to reduce both ourreviewing and production times after articles are accepted. Our targetfor acceptance to online publication is to reduce this from the currenteight weeks to four weeks.

In addition, our website for Pulmonary Circulation will includeinnovative tools to use and share articles, and we will continue to postarticles online immediately after they are accepted. We will broadenour readership through wider visibility of content and cross-promotion of articles with PVRI educational resources in order toincrease the likelihood of those articles being cited.

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Annual Report 2016

Our goal is to make our journal one of the world’s leading outlets forresearch on pulmonary vascular disease. We are ambitious in pursuingthis goal, and during 2016, we have laid the foundations for growth thatwe will build on during the years to come and we will be actively seekingto publish the highest quality articles. We look forward to receivingyour submissions.

Statistics

Number of Downloads of Pulmonary Circulation Articles by Year

Number of Downloads of Pulmonary Circulation Articles by Month,from October 2013 through October 2016

Articles Published to Date (all years). Total: 496 items: • Editorials – (37) 7%• Review – (101) 20%• Original Research – (242) 49%• Guidelines & Other – (68) 14%• Case Reports – (48) 10%

Accept and Reject RatesFrom January 2014 through October 2016, Pulmonary Circulationreviewed 211 Original Research articles, and 58 Case Reports.

• Original Research accepted: 171 (81%)• Original Research rejected: 40 (19%)• Case Reports accepted: 32 (55%)• Case Reports rejected: 26 (45%)

Top 15 Most Accessed Articles in 20161 Postural tachycardia syndrome is not caused by deconditioning,

Svetlana Blitshteyn, David Fries. Vol. 6, No. 3, September 2016.# Accesses 10,630

2 Perioperative pharmacological management of pulmonaryhypertensive crisis during congenital heart surgery, Nathan Brunner,Vinicio A. de Jesus Perez, Alice Richter, François Haddad, AndréDenault, Vanessa Rojas, Ke Yuan, Mark Orcholski, Xiaobo Liao. Vol. 4,No. 1, March 2014. # Accesses 977

3 Current concepts in the pathogenesis of chronic thromboembolicpulmonary hypertension, Daniel T. Matthews, Anna R. Hemnes. Vol. 6,No. 2, June 2016. # Accesses 959

4 Statement on pregnancy in pulmonary hypertension from thePulmonary Vascular Research Institute, Anna R. Hemnes, David G.Kiely, Barbara A. Cockrill, Zeenat Safdar, Victoria J. Wilson, Manal AlHazmi, Ioana R. Preston, Mandy R. MacLean, Tim Lahm. Vol. 5, No. 3,September 2015. # Accesses 918

5 Clinical classification in paediatric pulmonary arterial hypertensionassociated with congenital heart disease, Willemijn M. H. Zijlstra,Johannes M. Douwes, Mark-Jan Ploegstra, Usha Krishnan, Marcus T.R. Roofthooft, Hans L. Hillege, D. Dunbar Ivy, Erika B. Rosenzweig, RolfM. F. Berger. Vol. 6, No. 3, September 2016. # Accesses 853

6 Pulmonary hypertension in patients with heart failure and preservedejection fraction: differential diagnosis and management, NehalHussain, Athanasios Charalampopoulos, Sheila Ramjug, RobinCondliffe, Charlie A. Elliot, Laurence O’Toole, Andrew Swift, David GKiely. Vol. 6, No. 1, March 2016. # Accesses 827

7 Sildenafil in heart failure with reactive pulmonary hypertension(Sildenafil HF) clinical trial (rationale and design), Maya Guglin, NavinRajagopalan, Paul Anaya, Richard Charnig. Vol. 6, No. 2, June 2016.# Accesses 620

8 Transthoracic echocardiography in the evaluation of paediatricpulmonary hypertension and ventricular dysfunction, MartinKoestenberger, Mark K. Friedberg, Eirik Nestaas, Ina Michel-Behnke,Georg Hansmann. Vol. 6, No. 1, March 2016. # Accesses 562

9 Cardiac catheterisation in children with pulmonary hypertensivevascular disease: consensus statement from the Pulmonary VascularResearch Institute, Paediatric and Congenital Heart Disease TaskForces, Maria Jesus del Cerro, Shahin Moledina, Sheila G. Haworth,Dunbar Ivy, Maha Al Dabbagh, Hanaa Banjar, Gabriel Diaz, AlexandriaHeath-Freudenthal, Ahmed Nasser Galal, Tilman Humpl, SnehalKulkarni, Antonio Lopes, Ana Olga Mocumbi, G. D. Puri, BeyraRossouw, S. Harikrishnan, Anita Saxena, Patience Udo, Lina Caicedo,Omar Tamimi, Ian Adatia. Vol. 6, No. 1, March 2016. # Accesses 552

10 Cellular, pharmacological, and biophysical evaluation of explantedlungs from a patient with sickle cell disease and severe pulmonaryarterial hypertension, Natasha M. Rogers, Mingyi Yao, John Sembrat,M. Patricia George, Heather Knupp, Mark Ross, Maryam Sharifi-Sanjani, Jadranka Milosevic, Claudette St. Croix, Revathi Rajkumar,Maria G. Frid, Kendall S. Hunter, Luciano Mazzaro, Enrico M. Novelli,Kurt R. Stenmark, Mark T. Gladwin, Ferhaan Ahmad, Hunter C.Champion, Jeffrey S. Isenberg. Vol. 3, No. 4, December 2013.# Accesses 537

11 Protocol for exercise hemodynamic assessment: performing aninvasive cardiopulmonary exercise test in clinical practice, Natalia C.Berry, Agarwal Manyoo, William M. Oldham, Thomas E. Stephens,Ronald H. Goldstein, Aaron B. Waxman, Julie A. Tracy, Peter J. Leary,Jane A. Leopold, Scott Kinlay, Alexander R. Opotowsky, David M.Systrom, Bradley A. Maron. Vol. 5, No. 4, December 2015. # Accesses483

12 Novel signaling pathways in pulmonary arterial hypertension (2015Grover Conference Series), Keytam S. Awad, James D. West, Viniciode Jesus Perez, Margaret MacLean. Vol. 6, No. 3, September 2016.# Accesses 446

13 Unexplained exertional dyspnea caused by low ventricular fillingpressures: results from clinical invasive cardiopulmonary exercisetesting, William M. Oldham, Gregory D. Lewis, Alexander R.Opotowsky, Aaron B. Waxman, David M. Systrom. Vol. 6, No. 1, March2016. # Accesses 419

14 Echocardiographic parameters of right ventricular function predictmortality in acute respiratory distress syndrome: a pilot study, TrushilG. Shah, Subeer K. Wadia, Julie Kovach, Louis Fogg, Rajive Tandon. Vol.6, No. 2, June 2016. # Accesses 417

15 Mechanisms regulating endothelial permeability, Sukriti Sukriti,Mohammad Tauseef, Pascal Yazbeck, Dolly Mehta. Vol. 4, No. 4,December 2014. # Accesses 410

Pulmonary Vascular Research Institute

PVRI

International ScientificMeetings

Annual Report 2016

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Activities New to This ProgrammeThe title of this organisation includes the word‘research’ and to this end a new Task Force held itsfirst meeting, and was led by Mandy MacLean, aone day meeting that preceded the scientificprogramme. This meeting of the Pre-clinical &Molecular Task Force had a very full, successfulprogramme which facilitated communication andcollaboration between basic scientists. It isenvisaged that work facilitated by this Task Forcewill eventually feed into the Drug Discovery &Development Symposium. Other Task Forces met asusual, including the Paediatric & Congenital HeartDisease and the Women’s Health & Pregnancy TaskForce; two Task Forces among our most activegroups. It is anticipated that the high-levelconclusions of this and other Task Forces will bepublished in Pulmonary Circulation, as has occurredin the past.

The future of the PVRI rests with the young.Therefore we held the First Annual PVRI Meetingof the Committee for Young Clinicians andScientists, which will become an annual event.

The Annual Congress has been accredited by the EuropeanAccreditation Council for Continuing Medical Education (EACCME).

Day 1: Advances in Our Understanding of PVDWe tried to begin at the beginning, given the present understandingthat most of our ills, including pulmonary hypertension, originate inintra-uterine life. Kent Thornburg, a friend and colleague of Dr Barker,began by discussing the early roots of chronic disease. Nick Morrelladdressed the role of genetics. He has an interest in BMPRII, butacknowledged that other recently discovered genes can play a role inthe aetiogy and/or maintenance of pulmonary vascular disease.Evangelos Michelakis is interested in the metabolic basis ofpulmonary hypertension, starting with the Warburg Hypothesis andthen the role of the electron transport chain within the mitochondriaand the possibility of therapeutic intervention. Lastly, Brad Maronincorporated the molecular knowledge into an integrated, systemsapproach to pulmonary hypertension, emphasising the complexity ofthe mechanisms involved.

It was appropriate to consider whether the pulmonary hypertensioncommunity is moving in the right direction. Lewis Rubin summarisedthe current guidelines clearly and concisely while Majdy Idreesaddressed the difficult issue of how best to secure good quality carein developing countries. Pulmonary hypertension is frequentlyundiagnosed throughout the world and Ian Adatia spoke of his newapproach to the analysis of heart sounds using the non-invasive devicehe and his colleagues have just developed.

Having made the diagnosis, it remains challenging to assess thefunctional capacity/functional reserve of each patient. Difficulties inassessing the reserve of the right ventricle, particularly bytransthoracic echocardiography were discussed by Stefano Ghio andthe pros and cons of exercise studies were assessed in adults andchildren by Robert Naejie and Astrid Lammers respectively.

Turning to basic science, we were all excited by the possibility ofstudying pulmonary function using the human lung-on-a-chip model,presented by Donald Ingber who pioneered this technique. RalphSchermuly then questioned the relevance of the numerous rat, mouse,and pig models we all use to study human disease - a very helpful,critical appraisal.

Day 2During the second day we considered new frontiers in diagnosis,beginning by evaluating the role of imaging in the clinic, presented byDavid Kiely. We then considered the contribution of nuclear medicineand CT imaging, led by Horst Olschewski while Onno Spruijt askedhow repeated assessment of RV structure and function could helpin patient management.

A talk by Kurt Stenmark on the biology of the lung and cardiacstiffening helped put the clinical work into perspective as didthe experimental work by Lan Zhao 18FDG positron emissiontomography. Recognising the recent advances in echocardiographyMichele D’Alto reviewed its potentially useful role in differentiatingpre-from post-capillary PH.

PVRI 10th Annual World Congress in RomeReport 2016Sheila Glennis Haworth, Past President 2014-2016

The aim of this Annual Congress was to facilitate friendship, collaboration and interaction across the globe and to keep theprogramme relevant to all people. The speakers and chairmen came from all over the world. The highlights of the programmeare detailed below.

ROME CONGRESS FACTFILEDuration: 31/2 days

Content: 42 presentations, 42 discussions

Participation: 223 delegates from 28 different countries

Abstract submissions: 36moderated poster presentations

Additional meetings: 7 Task Force meetings

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Annual Report 2016

We cannot cure pulmonary vascular disease and therefore it isimperative to prevent it when it is possible to do so. One of thecommonest, if not the commonest cause of pulmonary vasculardisease worldwide is the Eisenmenger Syndrome, un-operatedcongenital heart disease. The burden, and therefore the cost, of thedisease is frequently unknown and so we heard a public healthperspective from Krishna Kunar from India. South America wasrepresented by Alexandra Heath from Bolivia and Maria AngelicaBinotto from Brazil while others from Saudi Arabia spoke of theirexperiences in a lively discussion led by Sir Magdi Yacoub andSheila G Haworth.

Day 3On day three we learnt about current therapies and considered futurepossibilities, beginning with an overview of current regimes from ArdiGhofrani and comments on the current use of combination therapyby Paul Hassoun. Mandy MacLean discussed the impact of gender andAnna Hemnes talked about pharmacogenetics, both striving toanswer the question, ‘will this drug improve my patient?’ Thinking ofthose patients who have become unresponsive to all currenttherapies, Damien Bonnet presented his promising findings on thePotts Shunt, a classic operation with a new application which showsgreat promise for some of our patients. In the developing worldprescribing current approved medicines with limited financialresources remains challenging, as we heard from BKS Sastry fromIndia.

The PVRI is anxious to push ahead and constantly evaluate ourpresent, clearly inadequate, treatment strategies. The session onadvancing our approach to medical therapy opened with Lewis Rubinconsidering new trial designs for PH, led to a lively debate chaired byJohn Newman and Pilar Escribano-Subias. The selection of newtherapeutic targets included immune targets (John Wort) andinfectious agents, mediators of inflammation in congenital heartdisease, recent work presented by Antonio Lopes. EvangelosMichelakis discussed targeting metabolic disturbances and thepotential of several metals, particularly iron, was assessed by MartinWilkins. Warning of potential interaction between treating anunderlying disease and treating PH, Nicolas Petrosillo commented onthe interaction of ante-retroviral drugs and PH specific therapies. Thecontribution of inflammation to the disease process is welldocumented and Allan Lawrie and Nick Morrell wondered ifantibiotics could be helpful therapeutic agents in PVD.

Day 4On day four we considered the emerging clinical challenges for thePH specialist, particularly the growing problem of PH in heart failure,a major challenge for the cardiologist. Stephan Rosenkranz and MarcoGuazzi spoke, respectively, of the diagnostic and therapeuticdifficulties. Paul Hassoun emphasised phenotypic differences in RVremodelling. It was left to Werner Seeger to discuss the scale of theproblem in lung disease and the indications for therapeuticintervention when PH is present.

Lastly, we joined the International Heart & Lung TransplantationSociety - a first joint symposium on Thrombo-embolic PH - in whichaetiology, diagnosis and, medical and surgical treatment werediscussed by Mark Toshner, Zhenguo Zhai, Andrea D’Armini andPaul Corris.

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Annual Report 2016

The 3rd PVRI Annual Drug Discovery &Development Symposium 2016Stuart Rich & John Newman

The 3rd Annual Drug Discovery & Development Symposium took place in Bethesda, DC, this year and proved an excellentwell-attended event. It attracted physician scientists, scientists and representatives from the pharmaceutical industryand drug regulatory bodies.

BETHESDA DRuG SYMPOSIuM FACTFILEDuration: 11/2 days

Participation: 112 delegates from science, pharma and regulatory bodies

Content: 12 presentations on new trials, new targets, new drugs

The Symposium remains true to its ideal, offering a unique comingtogether of all stakeholders involved in translating promisingmolecules into proven licensed therapies for clinical use within thefield of pulmonary vascular disease.

The meeting offered much more than this, however, with excellenttalks on potential new targets for therapy, including BMPR2,oestrogen and mitochondria. There was also a great demonstrationthat exercise-induced pulmonary hypertension is a real clinical entityleading to symptomatic breathlessness.

In 2017, the meeting will be held in Berlin and the PVRI would urgeyou all to keep an eye on our website and to register early.

This meeting offers a unique opportunity to participate in vibrantdebate and listen to excellent state-of-the-art talks on contemporaryand future therapeutic approaches to pulmonary hypertension.

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Annual Report 2016

PVRI 1st Annual Symposium for Young Investigators Overview2016Djuro Kosanovic, Mariola Bednorz & Michiel Alexander de Raaf

Blog by Michiel Alexander de Raaf:

When Djuro sent me an email that he had rented a castle for theweekend and asked me to join him, I had mixed feelings of surprise andexcitement.

Would this finally be the moment to push our scientific collaborativefriendship further? Perhaps I misjudged the situation a bit, I thought,especially when I noticed that I was not the only one to be invited.And then I understood. It was the PVRI Symposium for the Committeeof Young Clinicians & Scientists (CYCS). It was work. Science is,though, a game.

The first - and therefore historical - PVRI Annual Symposium forYoung Investigators was located at the Waldeck Castle in Germany.The castle is known to have had substantial influence on world history.In 1858 for example, Emma of Waldeck and Pyrmont was born there.She married the Dutch King William III who needed to stabilise hislegacy after his unsuccesful previous marriage to his niece Sophia ofWürttemberg. Emma gave birth to our later Queen Wilhelminaand after the death of William III, was regent when Wilhelmina wasstill a child. With this in mind, we tried to let the influence of Waldeckcastle be reflected in our support for evolving the PVRI.

The programme started on Friday afternoon with the welcoming andwas followed by a lovely dinner. The CYCS board discussed theagenda. Later that evening, we had a tour of the castle and saw thedungeons, prisons and well, which was dug by two prisoners whowere promised to be released if they found water! After 27 yearsand digging 140 meters, having lost their eyesight they eventuallyfound water. Upon release, one of the prisoners was so excited thathe died of a heart attack.

On Saturday, there was a CYCS plenary council meeting in themorning, addressing all ongoing topics, some of which were discussedimmediately and others postponed to the following day. In theafternoon we had a splendid social teambuilding event, which involvedleaving the castle via a cable track and then sailing on the lake. In theevening, after dinner, we started our scientific session which was full

of interactive discussions. In total we had 14 presentations (from the11 attendees) and covered a broad spectrum of present day cutting-edge science in the field of PVD.

On Sunday the day started with the second half of the CYCS plenarycouncil meeting, where we discussed the remaining topics. For meunfortunately, the Waldeck Symposium finished slightly earlybecause my wife, also a scientist, was on her way to a congress andI headed back home to continue my duties as a father. The othersremained and continued in the same spirit and ambiance as theprevious days, having the second part of the scientific sessionsclosed by a dinner at the castle.

Aside from the scientific achievement, there were other successes.Regarding the PVRI CYCS tasks, the timing of this retreat was superb,as we were able to discuss ongoing projects and start preparationsfor the Annual Congress in Miami. We had two ‘plenary councilmeetings’ (and one board meeting) to discuss and evaluate all ongoingprojects. I sincerely believe we made great progress and the topicsdiscussed at Waldeck will result in a more effective CYCS for a longtime to come. We were ableto produce and providesomething for the PVRI,which is an important benefitof the Waldeck retreat andcould also be capitalised uponin the future.

Regarding friendship, thisSymposium had a marvelousatmosphere; giving ample possibility to start and strengthenfriendships. This is already reflected in the increased dedication andactivity being experienced now by our members. All attendees are soenthusiastic that they are keen to make a possible future Symposiuman even greater success.

All attendees of the 1st PVRI Annual Symposium for YoungInvestigators at Waldeck, would like to acknowledge MariolaBednorz for the wonderful organisation of the historical meeting. ThisSymposium showed the strength of our CYCS and, personally, I feelvery fortunate to have had this experience.

1st YOUNG INVESTIGATOR SYMPOSIUM FACTFILEEvent: 1st Symposium for Young Investigators in PVD

Duration: 21/2 days

Participation: 12 participants from around the world

Content:Great science, great social programme

Beautiful rolling clouds surround the castle contrasting the dungeons below.

Pulmonary Vascular Research Institute

PVRI

Regional Task ForceReports

Annual Report 2016

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Annual Report 2016

Cardio-Pulmonary Acclimatisation and Adaptation toHigh-Altitude: from Physiology to Clinical Practice.The High-Altitude Task Force meeting took place on 24-28 September 2016, in Cholpon Ata (Lake Issyk-Kul),Kyrgyzstan.

Background and AimThe Issyk-Kul Leh Symposium took place on 24-28 September 2016,in the Karven Four Seasons Hotel on the shore of Lake Issyk-Kul,Kyrgyzstan. Its aim was to bring together the knowledge andinformation gathered from recent advances in high-altitude research.The Symposium followed the tradition of three previous high-altitudemedicine conferences held in Leh, Ladakh (India) from 2010 onwards.As in previous years, the conference was held in a highland area todraw attention to the needs of highland populations, as well asindividuals travelling to altitude. Additionally, this was the firstopportunity for high-altitude researchers from Central Asia to takecentre stage, be directly involved in the preparations for theconference as well as present their own original research fordiscussion with their colleagues from all over the world (UK, USA,India, Germany, Switzerland and others). Thus, throughout theSymposium, an atmosphere of constructive dialogue and maximumengagement of young researchers was maintained.

Research PresentationsAn international faculty of outstanding experts in various fields ofhigh-altitude physiology and medicine presented original researchand up-to-date reviews on selected topics.

Hypoxia and GeneticsThe first full day of the Symposium was opened with a presentationfrom a young researcher, Dr Eichstaedt (Heidelberg, Germany), ongenetic and phenotypic adaptation of populations to altitude. Morelecturers reported on genetic mutations in Kyrgyz highlanders,underlining the necessity of further research on this particularpopulation, since (compared to Andeans and Tibetans) it is a recentnewcomer to high-altitude; thus, processes of human adaptation tothis environment are a particularly interesting and important studysubject.

Response to Hypoxia at the Molecular LevelThis section also contained presentations of original studies, inparticular that of young researchers like Dr Hodson (Oxford, UK), onhypoxia-inducible factor (HIF) in mice models, which showed theimportance of HD2/HIF-2 in ventilator acclimatisation. Connected tothis was the session on hypoxia and pulmonary and systemiccirculation, with presentations by Japanese researchers (Dr Mizunoand team) on micro-RNA profiling of models of pulmonaryhypertension, and German colleagues (Dr Pak, University of Giessen),on the reaction of pulmonary vasculature to hypoxia.

Blood at AltitudeThis diverse session covered blood diseases and conditions connectedwith response of the blood to hypoxic conditions, such as those at highaltitude (Dr Muckenthaler, Heidelberg). It included a retrospective oftreatment of aplastic anemia in Kyrgyzstan by the highly respectedacademic. A. Raimjanov (Kyrgyzstan), to unique research on geneticdiseases that cause unregulated hypoxic response in normoxicconditions (Dr Gordeuk, USA/Russia), to studies of factors thatinfluence hemoglobin concentration (Dr Prchal, USA).

Good Clinical and Research Practice and EducationSeveral lectures were specifically dedicated to transferring knowledgeand experience of established researchers to their young colleagues.Specifically, methodology of study protocols, writing of scientificpapers (Dr Russi, Switzerland), as well as reports on successfuleducational programmes (Dr Schoch, Switzerland) were presented.

Mongolia

PVRI Central Asia & High-AltitudeReport 2016Max Gassmann, Qadar Pasha & Talant Sooronbaev

Kazakhstan

Turkmenistan

Uzbekistan

Tajikistan

Krygystan

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Annual Report 2016

Altitude Related Illness in High-Altitude TravellersSince millions of people travel to high-altitude every year for work orleisure, one of the most important aspects of this Symposium was theexchange of knowledge and discussion of the newest research onacclimatisation, as well as the detection and prevention of altitude-related illness (presentations by Dr Roach, USA; and Dr Burtscher,Austria).

COPD: Causes, Clinical Presentation at Low and High-AltitudeParticularly interesting were the presentations of original researchborne out of the recent Swiss-Kyrgyz cooperation projects, made byyoung researchers who participated in those studies (eg, M. Furianand M. Lichtblau, Switzerland, M.Mademilov and B. Estebesova,Kyrgyzstan).

Health Problems in HighlandersProblems related to insufficient adaptation of the human body toaltitude can severely impact the quality of life of high-altituderesidents. Presentations by Kyrgyz and international researchers (DrS.Ulrich, young scientists - B. Osmonov, S. Müller-Mottet) wereconcentrated on prevalence and treatment of diseases related toaltitude, such as high-altitude pulmonary hypertension (HAPH), aswell as other diseases tied to the lifestyle of highlanders (eg,prevalence of COPD and indoor pollution due to biomass heating).

Tuberculosis in Different SettingsTuberculosis, particularly multi-drug resistant (MDR) TB, is a graveproblem for many developing and emerging societies, and Kyrgyzstanin particular. Data presented by Dr Isakova (Kyrgyzstan) show thelarge extent of the problem, particularly in the penal system of thecountry, while Dr Schoch (Switzerland) shared valuable experience onearly diagnosis options for TB in developing countries.

Altitude Physiology in Early Life and During SleepDrs Reiss and Merkus, both established researchers from theNeonatology section of the Erasmus Medical Centre (Netherlands),provided their insight into the newest research on the effects ofaltitude on the foetus and the newborn child.

Physiology at the Extremes and Mountain MedicineAthletes and persons travelling to more extreme altitude conditionsdeserve special attention, which is why the conference also includedpresentations from researchers who have not only studied thoseconditions but experienced them personally. This included preventionof common health problems related to high-altitude (Dr Hess,Switzerland), relationships between doping and high-altitude training(Dr Suchy, Czech Republic) as well as a historical/philosophicalretrospective of climbing aids (Dr Kayser).

EducationOne of the main goals of this conference was to bring together youngand established researchers from all over the world, particularly fromdeveloping nations who do not get enough international exposure inmedical research. Thus, two special educational sessions were carriedout. The first was dedicated to methodology of research and papers,which would help young scientists create and publish their own workaccording to accepted international standards (by Dr Russi,Switzerland). The second included presentations on existingeducational programmes in developing countries, as well asexperience of improving the quality of diagnosis in low-incomecountries (by Dr Schoch, Switzerland). As noted above, specialattention was given to original research studies by young scientists.Among these, the winner of the international Young InvestigatorAward named after Academician M. Mirrakhimov was selected andpresented with a diploma (M. Furian, Switzerland).

Total Participants• Number of faculty members (intl.): 27 (Kyrgyzstan, Switzerland,

USA, UK, Germany, Canada, Netherlands, Spain, Czech Republic,Japan).

• Number of young researchers (total): 26.• Number of participants from Kyrgyzstan (total): 37.

Conclusions and OutlookOver four days, the Symposium brought together scientists ofdifferent interests tied to high-altitude medicine from a dozencountries across the globe. For Central Asia and for the field of high-altitude medicine, it became a unique event. Both the diversity of theparticipants, as well as the variety of topics were unprecedented:from pulmonary diseases and public health issues in highland andlowland, to specific physiological responses to hypoxia; fromtheoretical models and historical retrospectives, to the newestresearch, clinically relevant results and practically applicable advicefor patients at altitude.

In addition, the forum facilitated communication between young andestablished researchers, whilst the separate poster session helpedresearchers receive direct feedback on ways of improving theirresearch methodology and generated ideas for new research. Youngand established scientists were able to freely participate in scientificdiscussions, informal dialogue and team building events that arecrucial for improving intercultural communication, building lastingrelationships and establishing long-term partnerships. This had aninvaluable educational value for both researchers from developed anddeveloping countries alike; long term, this will undoubtedly lead tomore international cooperation and more quality medical research,particularly in areas that are severely lacking in resources, knowledge,and opportunities for young people.

We all are very grateful for the PVRI central office’s sponsorship,which has provided a cornerstone for future medical cooperation,particularly in the challenging environment of the Central Asianregion, and given us hope to see more unique research contributionsfrom that region at future conferences. 

Organising Committee of the Symposium• Prof. Talant M. Sooronbaev, MD (Bishkek, Kyrgyzstan).• Prof. Konrad E. Bloch, MD (Zurich, Switzerland).• Prof. Max Gassmann, DVM (Zurich, Switzerland and Lima, Peru).

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Annual Report 2016

The PVRI China Task Force continuesits active participation in organisingnational and international meetingsand education courses on pulmonaryvascular diseases (PVDs) in China, aiming toextend/update the understanding of pulmonaryembolism and pulmonary hypertension among Chinesephysicians, exchange clinical experiences and createfuture international collaboration opportunities. The PVRIChina Task Force encourages collaboration at internationallevel. Joint force research has resulted in severalcollaborative joint publications and promoted both clinicaland basic science PVDs research in China.

National Congress on Pulmonary Embolism and PulmonaryVascular Diseases and International Conferences of PulmonaryCirculation Disorders

The 8th National Congress on Pulmonary Embolism and PulmonaryVascular Diseases and the 6th International Conferences ofPulmonary Circulation Disorders, through the collaboration betweenThe Pulmonary Vascular Research Institute (PVRI) and the ChineseThoracic Society (CTS), were held in Suzhou, Jiangsu, China,8-10 April 2016. 

Leaders covered the most updated guidelines of venousthromboembolism (VTE) and pulmonary hypertension, especially theupdated antithrombotic and prevention of thrombosis guidelines andpulmonary hypertension guidelines, which provided the latestknowledge of diagnosis, treatment and prevention of pulmonaryvascular diseases. The PVRI also enhanced its educational programmeduring the meeting.

WorldPulmonaryHypertensionDay

May 2016:World Pulmonary

Hypertension Daywas celebrated on a

national scale. Physiciansfrom different cities organised

meetings to raise awareness of thedisease by staging various events involving both the scientificcommunity and the general public. A series of educational and socialactivities for pulmonary hypertension were held in Beijing with thesupport of the I-seek pulmonary hypertension advisory group. Morethan 200 multi-disciplinary physicians participated in the activitiesand the issues of health education, social support, medical insuranceand standardised treatment for Chinese pulmonary hypertensionpatients were discussed.

China Heart Congress August 2016: China Heart Congress. The day and a half pulmonaryvascular disease session was attended mostly by cardiologyphysicians. Professors Martin Wilkins, Xiansheng Chen, Lan Zhao andZhenguo Zhai gave lectures at the meeting, covering topics inadvances in PAH, as well as in congenital heart disease and medicaland surgical treatment of chronic thromoboembolisc pulmonaryhypertension.

The 15th National Conference of The Chinese Thoracic Society 22-25 September 2016: The 15th National Conference of TheChinese Thoracic Society was held in Xi’an, China. This meetingprovided a communication stage for physicians, scientists and otherhealthcare providers in China to ensure further progress in thediagnosis and treatment of pulmonary vascular disease including,pulmonary embolism and pulmonary hypertension.

PVRI China Report 2016Chen Wang , Zhengo Zhai, &Martin Wilkins

China

Professor Chen Wang

PVRI China Report 2016Chen Wang , Zhengo Zhai, &Martin Wilkins

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Annual Report 2016

Education programmeFebruary and July 2016: Two education programmes onstandardising the diagnosis and treatment of pulmonary vasculardiseases were published in the Chinese Medical Journal. Theseprovided updates on several important aspects, including diagnosisand treatment of pulmonary hypertension, diagnosis technology andstandard thrombolytic and anticoagulant therapy, as well as clarifyingstandardised operational procedure for imaging pulmonaryhypertension. More than 10 multidisciplinary physicians jointlyexplored and distributed some special points on the clinical practiceof evaluation and management of pulmonary embolism andpulmonary arterial hypertension.

A Multidisciplinary Consultation Platform for PE-PVDA multidisciplinary consultation platform for diagnosis andmanagement of pulmonary vascular diseases was set up in the China-Japan Friendship Hospital. Up to the end of October 2016, more than250 complicated PVD patients were discussed. This platformprovides a good support for individualised diagnosis and treatment ofpulmonary embolism and pulmonary hypertension. The platformalso provides excellent learning and communication opportunitiesfor physicians.

The Pulmonary Hypertension Academy The Pulmonary Hypertension Academy, a multicentre andmultidisciplinary education platform for diagnosis and managementof pulmonary vascular diseases, was set up in Beijing China. TheAcademy includes: China-Japan Friendship Hospital, Peking UnionHospital, Fuwai Hopsital, Anzhen Hospital, Chaoyang Hospital andBeijing Hospital and it provides excellent learning and communicationopportunities for young physicians interested in pulmonaryhypertension.

The China Day of ERSThe China Day of ERS was held in London in September 2016 withmore than 500 multidisciplinary physicians, including some physiciansfrom ERS and CTS in attendance. The aim was to explore China’sclinical practices of respiratory and pulmonary vascular diseases.  DrZhenguo Zhai gave a talk about the progress of pulmonary embolismand pulmonary vascular diseases in China and detailed discussion ofthe cooperation also took place.

Major publications• Zhang S, Zhai Z, Yang Y, Zhu J, Kuang T, Xie W, Yang S, Liu F, Gong

J, Shen YH, Wang C. Pulmonary embolism risk stratification byEuropean Society of Cardiology is associated with recurrentvenous thromboembolism: Findings from a long-term follow-upstudy. Int J Cardiol. 2016 Jan 1;202:275-81.

• Qiu J, Xie W, Zhai Z, Wan J, Wang C. Metastatic synovial sarcomaof lung mimicking pulmonary embolism and deep venousthrombosis. Thorax. 2016 Nov 4. pii:thoraxjnl-2016-209029. doi:10.1136/thoraxjnl-2016-209029. [Epub ahead of print] 

• Wang C, Zhai Z, Yang Y, Cheng Z, Ying K, Liang L, Dai H, Huang K,Lu W, Zhang Z, Cheng X, Shen YH, Davidson BL; China NationalVenous Thromboembolism (VTE) Study Group. Inverserelationship of bleeding risk with clot burden during pulmonaryembolism treatment with LMW heparin. Clin Respir J. 2016Sep;10(5):596-605. doi: 10.1111/crj.12262. PubMed PMID:25619125.

• Wang W, Liu J, Yang YH, Zhai ZG, Wang C, Wang J. Red celldistribution width is increased in chronic thromboembolicpulmonary hypertension. Clin Respir J. 2016 Jan;10(1):54-60. doi:10.1111/crj.12181. PubMed PMID: 24989160.

• Gong J, Zhai Z, Yang Y, Zhang Z, Zhang S, Xie W, Kuang T, Wang C.[Analysis ofclinical relevant bleeding events in the elderly patientswith acute pulmonary thromboembolism after treatment].Zhonghua Yi Xue Za Zhi. 2015 Nov 24;95(44):3588-92. Chinese.PubMed PMID: 26813370.

• Zou L, Xu X, Zhai Z, Yang T, Jin J, Xiao F, Wang C. Identification ofdownstream target genes regulated by the nitric oxide-solubleguanylate cyclase-cyclic guanosine monophosphate.

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Annual Report 2016

PVRI Eastern Mediterranean & SaudiArabia Report 2016Paul Hassoun & Majdy Idrees

Number of membersTotal local/regionalmembers was 162.

Educational events/meetingsThe following scientific and educational activities took place in 2016:• The 9th Annual Conference for EMR (SAPH 2016).

- February 2016 at Amman, Jordan.• The 1st CTD-PAH meeting for rheumatologists.

- November 2016, Riyadh, KSA.• Awareness Day for PH for internist & family physicians.

- March 2016, Riyadh, KSA.- June 2016, Jeddah, KSA.- June 2016, Madinah, KSA.- September 2016, Dammam, KSA.

• Awareness Day for PH for the public (PH Annual Day).- May 2016, Jeddah, KSA.

• Central Asian PH meeting (in collaboration with SAPH/EMR).- September 2016, Issyk-Kul, Kyrgyzstan.

Regional activities• PHUTURE programme for future PH physicians (in collaboration

with Bayer).- February 2016, Abu Dhabi, UAE.- April 2016, Kuwait City, Kuwait.- June 2016, Cairo, Egypt.- November 2016, Muscat, Oman.

Research (current research initiatives)• The prevalence of PH in sickle-cell disease in the Eastern

Provence in Saudi Arabia.• Inhaled Iloprost in severe pulmonary hypertension in COPD

patients.• Genetic studies in Saudi patients with congenital heart disease.• Bronchiectasis and PH in Saudi patients.• Prevalence of PH in the high altitude in Saudi Arabia.• Prevalence of CTEPH in Saudi Arabia.• Phenotyping of pulmonary hypertension.• PAH in scleroderma patients in Saudi Arabia.• PAH in CHD in Saudi patients.• Morbidity and mortality of right heart catheterisation in a

tertiary care centre in Saudi Arabia.• Contribution in the PVRI consensus statement for the

management of pulmonary hypertension in the developingworld.

Other initiatives• SAPH/EMR is working on establishing a further collaboration

with PHA Europe and PHA Canada.• SAPH/EMR is also planning to expand its collaboration and share

experience through working with PHA South Africa and manyearly initiatives/groups in North African countries.

• SAPH has established a partnership with the Central Asian PHgroup (CAPH).

Summary of activities for 2017• Organise the 10th Annual Joint (SAPH/PVRI) meeting in Dubai,

UAE, 2-4 February 2017. For more information about thismeeting, please visit the conference website(www.saph2017.com) or the SAPH website (www.saph.med.sa).

• Continue holding the paediatric PH master class in February andthe adult PH master class in October 2017.

• Continue holding frequent awareness days in different areas in theEMR region.

• Continue to participate in the international PH annual day on 5May 2017.

Goals for year 2017SAPH/EMR is planning to continue expanding its activities . It is nowrecognised regionally and, increasingly, internationally for its servicesin the field of PH.

SAPH will continue seeking to increase the number of activemembers, improving awareness and creating a positive environmentbetween physicians and healthcare providers interested in the field.Attracting more nurses, paramedics and patients is now SAPH’smain aim.

Lebanon

Israel

Jordan Kuwait

QatarEgypt

Iraq

Syria

Turkey

Iran

United ArabEmirates

Oman

Yemen

Saudi ArabiaBahrain

PVRI Eastern Mediterranean & SaudiArabia Report 2016Paul Hassoun & Majdy Idrees

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Annual Report 2016

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Annual Report 2016

A meeting, firmly supported by the PVRI, was held in Pavia,Italy, at the end of October 2016. Located in Northern Italy’sLombardia region, Pavia is a small town and home to afamous university founded in 1931.

Over the past few years, the Heart Failure and PulmonaryHypertension Centre of the Cardiology Division of the S. MatteoHospital in Pavia has been leading a regional network, which includesall specialists who look after patients affected by this rare andsevere disease.

The aim of the meeting was to increase awareness of pulmonaryhypertension and to strengthen cooperation among the medical staffof all Lombard centres who work in this field.

Distinguished international speakers who participated were:Prof. Paul Corris, President of the PVRI, Dr Juan Barberà fromBarcelona, Dr Robert Naeije from Brussels, Dr Luke Howard fromLondon and Dr Henning Gall from Giessen. Italian speakers includedProf Marco Guazzi from Milano, Prof. Dario Vizza from Rome andDr Michele D’Alto from Naples.

The speakers focused on the diagnostic difficulties and thetherapeutic possibilities both in Group 2 and in Group 3 pulmonaryhypertension. In addition, the latest randomised, placebo-controlledclinical trials were also discussed in depth. Preliminary data from theregional registry, which so far includes nearly 900 patients, werepresented by Dr Stefano Ghio, Laura Scelsi and Claudia Raineri fromS. Matteo Hospital.

The meeting in Pavia was also an excellent opportunity to discuss andto plan future patho-physiological research to be shared by theEuropean Task Force of the PVRI.

The Task Force is, in fact, firmly committed to sharing researchobjectives and addressing patho-physiological issues which cannot bepursued by a single centre. It is acknowledged that a multi-centreapproach has to be adopted, which is more in line with the objectivesin pharmacological trials.

Norway

Sweden

UnitedKingdom

Ireland

Portugal

Greece

Serbia

Croatia

Spain

Poland

France

Austria

Germany

Netherlands

Belgium

Switzerland

Italy

CzechRepublic

PVRI Europe Report 2016Stefano Ghio & Dario Vizza

PVRI Europe Report 2016Stefano Ghio & Dario Vizza

Denmark

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The Task Force has set up a PH club, which meets quarterlyin Hyderabad and Chennai, to discuss cases of interest.

On 5 May 2016, World PH Day, a series of webinars was held. TheTask Force also conducted a PH education programme and introducedyoga training in Hyderabad hospital. While it may not be scientificallyproven, many patients felt better and some are now attending, undersupervision, regular yoga sessions - this could be called rehabilitation.

On 2 and 3 July, a national level webinar took place. With the supportof the PVRI and the active support of Cipla, the Task Force conducteda national level webinar on 2 July 2016 in Chennai and on 3 July 2016in Mumbai. In addition, the India PVRI Task Force meeting was asuccess.

Nearly 270 doctors attendedand a total 650 doctors eitherlogged in online or attendedwebcast meetings at satellitecentres.

Dr Rubin from the USA andDr Gerhard Diller from Germanywere the international faculty.

There were nearly 20 Indian faculty and a widevariety of topics related to the practice of PH in India were covered.It was much appreciated with lively audience participation.

Annual Report 2016

India

Sri Lanka

PVRI India Report 2016BKS Sastry

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Annual Report 2016

PVRI North AmericaReport 2016Kurt Stenmark & Stephen Archer

The primary focus for this past year has beenthe planning of the PVRI 11th Annual WorldCongress in Miami, January 2017. Drs Archerand Stenmark, along with colleagues BrianGraham, Mark Ormiston, Brad Maron, andThenappan Thenappan have put together anextensive and exciting programme. Prestigiousspeakers from both North America and Europewill be attending.

The conference will cover everything from the newest, basicscience discoveries to translational and clinical state-of-the-art talks.There will be debates on clinically important topics and opportunitiesfor young investigators to present at highly interactive electronicposter sessions. This will facilitate interactions between both seniorand junior investigators.

The PVRI leadership has also worked with and acted as the liaison tothe Critical Care and Cardio-Pulmonary Research (3CPR) council ofthe American Heart Association (AHA). The long-term goal is tobetter integrate the work going on among these two importantorganisations. There will be a presentation by Dr Brad Maron at thePVRI 2017 Miami meeting describing the immediate and long-termgoals of this affiliation.

PVRI Get-Together 2016 In addition to the above, the North America Task Force organisedthe annual PVRI Get-Together 2016 at the ATS, which was held on17 May in San Francisco.

This social event was a great success, with 83 delegates attendingfrom around the world.

United States of America

Canada

PVRI North AmericaReport 2016Kurt Stenmark & Stephen Archer

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Annual Report 2016

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Annual Report 2016

Activities in 2016The Tracking Outcomes and Practice in Paediatric PulmonaryHypertension (TOPP 2) Registry on Pulmonary Hypertension inChildren was held in Chicago on 5 November 2016.

The Task Force gave a presentation on pulmonary hypertension inchildren at altitude and specifically on PH at moderate altitude (2.640meters over sea level). There were some interesting results related

to the effect of hypobaric hypoxia on epidemiology, pathogenes,diagnostic approach and treatment. Emphasis was made on theimportance of early diagnosis to avoid the remodelling of pulmonaryvasculature (40% of patients diagnosed this year were three years oldand younger).

It is interesting to note the frequency of PH in children at altitude:23 new patients registered in the last 12 months: 19 patients are stillregistered to date, one died before catheterism and therefore notregistered; and four are awaiting catheterism.

Altitude related PH pertains to Group 3 of Nice Classification(as Hypoxia). However, as for hypobaric hypoxia, the categorisationof PH hypertension at altitude mayneed to be considered as a specialsub-group as it differs to otherpathologies of Group 3 but also relatesto pathologies in Groups 1, 5 and,possibly, 2.

Outlook for 2017March 2017The Second Edition of the textbookCARDIOLOGÍA PEDIÁTRICA (inSpanish) will be launched in March2017 during a small symposium onpaediatric cardiology.

The textbookwill comprise75 chapters,five dedicatedto pulmonaryhypertension,and  will havearound 1,100 pages.

Chapters related topulmonaryhypertension will be: • Development of Pulmonary

Vasculature: Dr MarleneRabinovitch (USA).

• Persistent PulmonaryHypertension of thenewborn: Dr John Kinsella(USA).

• Idiopatic PulmonaryHypertension: Dr JulioSandoval (México).

• Pulmonary Hypertensionand Congenital HeartDisease: Dr MauriceBeghetti et al(Switzerland).

• The Children withPulmonaryHypertension ataltitude: Gabriel Díaz(Colombia).

Additionalcontributions by PVRIparticipants:• Dr Dunbar Ivy:

Transposition of thegreat arteries.

• María Jesús del Cerro:Congenital anomalies of coronaryarteries.

• Ian Adatia: Atrioventricular septal defects.

Guatemala

Honduras

Nicaragua

Costa Rica

Panama

Ecuador

Columbia

Venezuela

Brazil

Bolivia

Paraquay

Chile

Argentina

Uraguay

Mexico

Peru

Guyana

Suriname

French Guiana

PVRI South AmericaReport 2016Gabriel Diaz & Dora Haag

PVRI South AmericaReport 2016Gabriel Diaz & Dora Haag

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Annual Report 2016

April 2017

The Second Latin American Symposium on Pulmonary Hypertensionin Children (Segundo Simposio Latinoamericano sobre HipertensiónPulmonar en Niños) will be held in Buenos Aires 20-21 April 2017with the support of the PVRI.

The main objective of the event is to analyse the problem in LatinAmerica; round tables and conferences will be developed by differentprofessionals of the world. On the morning of 20 April 2017 twosuccessive didactic and interactive workshops of non-invasivediagnosis and cardiac catheterisation will be held respectively.

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Annual Report 2016

The past few years have seen the efforts of the South Africa& Sub-Saharan Africa Task Force produce pivotal datapublished in highly respected journals, which has providedextensive insight into the incidence, prevalence and natureof pulmonary hypertension in Sub-Saharan Africa, thushighlighting the importance of this clinical problem to theregion.

The imperative now is to translate this data into better care for ourpatients, irrespective of the great differences in access tosophisticated medical care and vast disparity in economic resources.

A step toward a more united approach to the management ofpulmonary hypertension has been made over the past two years withthe establishment of the Working Group for Pulmonary Hypertensionin South Africa, which was the first attempt of the pulmonology andcardiology community at a formal collaborative approach.

Publication of recommendations in the South African Medical Journalis aimed as a first step toward endorsement of the ESC/ERS 2015Guidelines in a form adapted to local conditions and, at the same time,broadening the awareness of, and improving access to, better medicalcare for patients with pulmonary hypertension.

While the above efforts were conducted independently of the PVRI,or indeed any other formally constituted organisation, there is apressing need for and eagerness to have collaborative input over andabove that of the many individual members of the international PHcommunity who have been so willing to assist our efforts thus far.

Congratulations to Karen Sliwa-Hahnle

In June 2016, during the World Congress of Cardiology andCardiovascular Health in Mexico, Professor Karen Sliwa-Hahnlewas elected President Elect of the World Heart Federation.

We would like to thank Karen for her enthusiasm, hard work andcommitment as the previous Task Force leader.

Published Data

PVRI South Africa & Sub-SaharanAfrica Report 2016Paul Williams

Zambia

Tanzania

MozambiqueZimbabwe

Botswana

South Africa

Angola

Namibia

PVRI South Africa & Sub-SaharanAfrica Report 2016Paul Williams

WORLD HEART FEDERATION

INTERNATIONAL JOURNAL OFCARDIOLOGY

SAMJ

THELANCET.COM

EUROPEAN JOURNAL OF HEARTFAILURE

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The South East Asia Task Force is a new geographical TaskForce and has only been established since the end of 2016.  

The Task Force leaders are already engaging interested physicians inMalaysia and have established contact in the Philippines.

2017 Activities

The first regional Task Force meeting will be held on 21 - 23 July 2017with the Malaysian Thoracic Society at their Congress.

The Task Force, with the support of the PVRI, will organise a CTEPH-related Pre-Congress workshop on 20 July, which has a working titleof Acute PE to CTEPH: State of the Art Management.

Annual Report 2016

PVRI South East Asia Report 2016Helmy Haja Mydin & Roslina Abd Manap

Singapore

Malaysia

Pulmonary Vascular Research Institute

PVRI

Disease & Speciality Task ForceReports

Annual Report 2016

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Annual Report 2016

PVRI Exercise Report 2016David Systrom & Aaron Waxman

Achievements So FarAt the Rome PVRI Congress in January 2016, the Task Forcemembership agreed on a broad framework of a consensus statementon exercise pulmonary hypertension as a disease. The documentoutline is as follows:

• ePH is associated with exertional intolerance.

• ePH substantively affects exercise tolerance through decreasesin oxygen delivery and its peripheral utilisation.

• Transpulmonary flux of biomarkers suggest ePH is associatedwith an early vasculopathy.

• Event free survival is reduced and untreated ePH.

• Treatment of ePH with pulmonary vasodilators improvessymptoms, exercise capacity and long-term outcome.

PublicationsNew guidelines for standardised invasive exercise testingBerry NC, Manyoo A, Oldham WM, Stephens TE, Goldstein RH,Waxman AB, Tracy JA, Leary PJ, Leopold JA, Kinlay S, Opotowsky AR,Systrom DM, Maron BA. Protocol for exercise hemodynamicassessment: performing an invasive cardiopulmonary exercise test inclinical practice.

Pulm Circ. 2015 Dec;5(4):610-8. doi: 10.1086/683815. Review. PMID: 26697168

The first large study of the upper limits of normal for exercisepulmonary hemodynamics in a ‘true’ normal populationOliveira RKF,Agarwal M,. Tracy JA, Karin AL, Opotowsky AR, WaxmanAB, Systrom DM. Age-related upper limits of normal for maximumupright exercise pulmonary hemodynamics.

Eur Resp J. DOI: 10.1183/13993003.01307-2015 Published 17 December 2015

A paper describing early compliance changes in precapillaryexercise PH which differ from heart failure preserved ejectionfractionOliveira RK, Waxman AB, Agarwal M, Badr Eslam R, Systrom DM.Pulmonary hemodynamics during recovery from maximumincremental cycling exercise. Eur Resp J 2016 Apr 28. pii: ERJ-00023-2016. doi: 10.1183/13993003.00023-2016. [Epub

ahead of print]

Goals/Aims For 2017• Better understanding of the functional relationship between

borderline and exercise pulmonary hypertension.

• Effect of pulmonary vasodilator treatment on exercise pH.

• Long-term outcomes of untreated ePH.

• Deep phenotyping of ePH, including imaging and transpulmonaryflux of biomarkers (metabolomics, proteomics, transcriptomicsand coagulomics).

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Annual Report 2016

PVRI Imaging Report 2016David Kiely & Andy Swift

Strategy for 2017-19

Phase 1To develop a comprehensive position statement for publicationin Pulmonary Circulation.  The production of this document bringstogether experts in the field of imaging and pulmonary hypertensionand is expected to act as a springboard for further collaborations. 

The position statement includes the following sections with a numberof work-streams in each section with all imaging task force membersinvolved:

• Introduction: overview current pulmonary hypertensionlandscape and potential role for imaging.

• Review of established and emerging imaging modalities withrespect to their use in the assessment of pulmonary vasculardisease.

• Focused sections covering clinical challenges, radiologycontroversies, technical aspects and developing networks ofimaging excellence and sharing of expertise.

• Diagnostic algorithms include state of the art approaches toimaging and algorithms based on image availability.

Phase 2Plan to develop educational interactive online cases.

Phase 3Roll out of centres of PH imaging excellence.

Goals for 2017-2018

January 2017Meeting of the Task Force in Miami  on Friday 27 January 2017, (2pmto 5pm) to review individual sections of the position statement.

• Lead of each work-stream or deputy to present core findings at thePVRI Imaging Symposium to finalise the scope of the document.

January 2017 to June 2017Refine the initial draft of position statement prior to voting onrecommendations and subsequent refinements prior to submissionto Pulmonary Circulation.

October 2017Submit position statement to Pulmonary Circulation.

January 2018Present final guidelines in dedicated Imaging at the PVRI Symposiummeeting to be launched.

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Annual Report 2016

2016 Activities

• Paediatric Pulmonary Hypertensive Vascular Disease organisedby Paediatric Task Force members, and with major contributionsfrom Paediatric Task Force members.

• 14-17 January 2016 Paediatric & Congenital Heart Disease TaskForce Meeting during the Annual PVRI Meeting, Rome (Italy). 

• 10-12 March 2016 9th International Conference Neonatal &Childhood Pulmonary Vascular Disease. San Francisco, CA , USAOrganised by Jeff Fineman and Ian Adatia.

• 24 June 2016 First Paediatric and Neonatal PulmonaryHypertension Update. Kokilaben Dhirubai Ambani Hospital,Mumbai, in association with IAP Mumbai and PVRI, organised byPrashant Bobhate and Shreepal Jain.

• 26 June 2016 Amrita Paediatric Pulmonary Hypertension Updateorganised by Shine Kumar and KK Kumar, Cochin, Kerala.

• 29 October  2016 International Symposium on PaediatricPulmonary Hypertension, Clinica Shaio, Bogotá, organised by LinaCaicedo. Participation of Humberto Garcia Aguilar, Rachel Hopper,Gabriel Diaz, Maria Jesus del Cerro.

2016 Achievements

• Publication of consensus document: “Cardiac catheterisation inchildren with pulmonary hypertensive vascular disease: consensusstatement from the Pulmonary Vascular Research Institute,Paediatric and Congenital Heart Disease Task Forces”.

• Maria Jesus del Cerro, Shahin Moledina, Sheila G. Haworth,Dunbar Ivy, Maha Al Dabbagh, Hanaa Banjar, Gabriel Diaz,Alexandria Heath-Freudenthal, Ahmed Nasser Galal, TilmanHumpl, Snehal Kulkarni, Antonio Lopes, Ana Olga Mocumbi, G. D.Puri, Beyra Rossouw, S. Harikrishnan, Anita Saxena, Patience Udo,Lina Caicedo, Omar Tamimi, Ian Adatia. Pulmonary Circulation2016; 6(1).

• We welcomed new members to the Paediatric & Congenital HeartDisease Task Force: Drs Rachel Hopper (US), Sadia Quyam (UK),Aigerim Tobayeva (Kazhajistan), Jeff Feinstein (US), Stephanie Siehr(US) .

2017 Goals

• To celebrate the Annual Paediatric & Congenital Heart DiseaseTask Force Meeting, on 25 January 2017, in Miami.

• To publish a new consensus document: Management of PulmonaryVascular Disease in Bronchopulmonary Dysplasis.

• To collaborate in the organisation of the Second Latin Meeting onPaediatric Pulmonary Hypertension, to be held in Buenos Aires on19-20 April 2017.

• To celebrate on 9-11 March 2017 the 10th InternationalConference Neonatal & Childhood Pulmonary VascularDisease,   San Francisco, CA, USA organised by Ian Adatia andJeff Fineman.

To update the Panama Paediatric Classifications on Functional Class and Diagnostic Phenotypes.

PVRI Paediatric & Congenital HeartDisease Report 2016Ian Adatia, Maria Jesus del Cerro & Steven Abman

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Annual Report 2016

PVRI Pre-Clinical & Molecular Science Report2016Mandy MacLean

The Pre-Clinical & Molecular Science Task Force met for the first time at the PVRI Congress in Romein January 2016.

They held a well-attended one-day workshop discussing pre-clinicalanimal models and their relevance to disease, taking sex into account.Also discussed were pre-clinical studies on biomarkers and noveltherapeutic approaches.

There was a lively discussion and a poster session over lunch. Theworkshop was co-organised by Mandy MacLean and Duncan Stewart.

During 2016, the Pre-Clinical & Molecular Science Task Force hasbeen organising another one-day workshop for Miami 2017, whichwill be run with the Women’s Health Task Force to discuss issues offemale sex in PAH. This will be run by Mandy MacLean and AnnaHemnes.

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Annual Report 2016

Members

• Angela Bandeira - University of Pernambuco, Brazil• Ghazwan Butrous - University of Kent, United Kingdom• Alexi Crosby - University of Cambridge, United Kingdom• Ana Lucia Coutinho Domingues - University of Pernambuco, Brazil• Rita Ferreira - University of Pernambuco, Brazil• Brian Graham - University of Colorado, United States• Essam Hamoda - Kuwait• Rahul Kumar - University of Colorado, United States• Nicholas Morrell - University of Cambridge, United Kingdom• Rubin Tuder - University of Colorado, United States

Looking Back at the Past Year

Members of the Schistosomiasis Task Force had multiple publicationsrelevant to the mission of the Task Force, including (but not limitedto):

• Leite LA, Ferreira Rde C, Hatzlhofer BL, Correia MC, Bandeira ÂP,Owen JS, Lima VL, Domingues AL, Lopes EP. Portal VeinThrombosis Associated with Protein C Deficiency and ElevatedFactor VIII in Hepatosplenic Schistosomiasis. Blood CoagulFibrinolysis. 2016 Mar;27(2):210-2.

• Leite LA, Pimenta Filho AA, Ferreira Rde C, da Fonseca CS, dosSantos BS, Montenegro SM, Lopes EP, Domingues AL,Owen JS, Lima VL. Splenectomy Improves Hemostatic andLiver Functions in Hepatosplenic SchistosomiasisMansoni. PLoS One. 2015 Aug 12;10(8):e0135370.

• Barbosa CS, de Souza Gomes EC, Campos JV, de Oliveira FJ, daSilva Mesquita MC, de Oliveira EC, Domingues AL. Morbidity ofmansoni schistosomiasis in Pernambuco-Brazil: Analysis on theTemporal Evolution of Deaths, Hospital Admissions and SevereClinical Forms (1999-2014). Acta Trop. 2016 Jul 2;164:10-16. doi:10.1016/j.actatropica.2016.06.024.

• Crosby A, Soon E, Jones FM, Southwood MR, Haghighat L, ToshnerMR, Raine T, Horan I, Yang P, Moore S, Ferrer E, Wright P, OrmistonML, White RJ, Haight DA, Dunne DW, Morrell NW.Hepatic Shunting of Eggs and Pulmonary Vascular Remodellingin Bmpr2(+/-) Mice with Schistosomiasis. Am J Respir Crit CareMed. 2015 Dec 1;192(11):1355-65

• Kumar R, Mickael C, Chabon J, Gebreab L, Rutebemberwa A,Garcia AR, Koyanagi DE, Sanders L, Gandjeva A, Kearns MT,Barthel L, Janssen WJ, Mauad T, Bandeira A, Schmidt E, Tuder RM,Graham BB. The Causal Role of IL-4 and IL-13 in Schistosomamansoni Pulmonary Hypertension. Am J Respir Crit Care Med.2015 Oct 15;192(8):998-1008.

PVRI Schistosomiasis Report 2016Brian Graham

Annual Report 2015

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Annual Report 2016

PVRI Women’s Health & PregnancyReport 2016Anna Hemnes & Barbara Cockrill

The Women’s Health and Pregnancy Task Force met during the PVRI Annual Meeting in Rome and discussed potentialfuture directions for our group.

The Task Force decided to co-sponsor a Pre-Congress Symposiumwith the Pre-Clinical & Molecular Science Task Force.

This has been planned with input from both Task Forces. The meetingpromises to be an exciting, thought-provoking discussion of the roleof sex hormones at the intersection of basic science and humandisease in pulmonary hypertension.

The Task Force is working on a book based on our recent consensusstatement published in Pulmonary Circulation that is beingspearheaded by Tim Lahm.

Finally, the Task Force is considering future studies to promotewomen’s health with regard to the pulmonary circulation in the future,as well as surveys of exposures and practice patterns.

Pulmonary Vascular Research Institute

PVRI

www.pvri.info

The PVRI is a Registered Charity in the uK, No: 1127115. In the uSA, the PVRI is registered as a not-for-profit organisation (501c3) in the State of Illinois (IN26: 0420959).

Today’s work, tomorrow’s possibility...