The 4th annual Joint Pulmonary Hypertension conference in Dubai, April 19th-22nd 2011, was the continuation of a line of annual meetings organized by the Saudi Association of Pulmonary Hypertension (SAPH) and the Eastern Mediterranean Taskforce of the PVRI. The first meeting was held in 2008 in Dubai, followed in 2009 by a meeting in Casablanca, Morocco. In 2010 SAPH hosted the meeting in Shram el Sheik, Egypt whilst this fourth meeting was planned to take place in Beirut. However, due to the political instability in the area the organizer was forced to change the location to a more stable, easily accessible city: Dubai. This was a positive move as all interested parties were able to attend the meeting as planned.
These annual conferences have proved a very successful venture as the topics as well as the attendees have increased each year. At this 4th meeting, over 140 attendees from various parts of the Middle East were in attendance. Similarly, an increase in the level of discussion and general awareness has become noticeable, both of which are strong indicators that the joint SAPH and EMR taskforce goal of enhanced awareness of pulmonary hypertension is being met. For further reflection on this topic please see Managing Director Ghazwan Butrous' blog at http://pvri.info/blogs/butrousg
 

The 4th annual Joint Pulmonary Hypertension conference started with a keynote talk by Professor Ghazwan Butrous on the role of Islamic scientists and physicians in the development of pulmonary hypertension, a topic which is addressed further in the Pulmonary Vascular Medicine History Initiative section of the first issue of the PVRI journal Pulmonary Circulation. The discussion was very lively with 60 people present for the meeting, and a similar atmosphere continued during cocktails and dinner.
 
The following morning the meeting began with a warm welcome from the chairpeople of the session, Dr. Mohamed Al Hajjaj and Dr Mirza Al Sayegh from UAE. The word was given to Dr Majdy Idrees, head of SAPH and organizer and chairman of this meeting. Dr Idrees initiated this series of meetings and has been very active both within SAPH, and as vice-president of the East Mediterranean region of the PVRI, a role he shares with Dr Paul Hassoun.  Dr Idrees started the discussion by reminding the attendees of the SAPH mission, which states that "we committed ourselves to be the pilot group to take the lead in the management of pulmonary hypertension in the region, and to serve our patients by providing optimal medical service, support and care."  He reiterated that SAPH is a national not-for-profit organization, and is currently supported by the Saudi Thoracic Society. SAPH's main goal is to address issues related to the training and education of health care professionals in the area of pulmonary vascular diseases. SAPH is a partner of the PVRI and has in the past worked with the PVRI to enhance its collaboration with other international regional associations, and will continue to do so.
 In the last five years, SAPH has experienced fantastic growth, going from 6 to 74 members including pulmonologists, cardiologists, intensivists, pediatricians and rheumatologists.
 
Dr Idrees presented the main achievements of SAPH as a summary of three major topics: Firstly, it began and continued the tradition of the Annual Meeting. Secondly, SAPH published the Saudi guidelines for pulmonary hypertension, and thirdly, it achieved increased awareness in the kingdom of Saudi Arabia through a series of initiatives. Similarly, Dr Idrees summarized the initial findings from the pulmonary hypertension SAPH registry. Many centers have contributed to this registry and the number of patients is growing. Dr Idrees presented a small group of patients representatives from the Riyadh area, whose analysis was available at the time of presentation.  Within this data, two main issues were highlighted; namely, how the diagnosis was performed, and when the patient was presented to the specialist centers. Unfortunately, the data was found to represent a trend of an 18 month--or more--delay before diagnosis. Obviously, this is not at all optimal and is in need of evaluation. This is a very important finding that can hopefully help guide the policy for increasing awareness for early diagnosis and treatment. 
Professor Gerald Simmonneau, from France, followed this enlightening presentation with his own on the clinical classification of pulmonary hypertension and the recent revision of Dana Point. Dr Simmonneau stressed the importance of including recent clinical findings and advances in clinical classifications, and the need to clarify areas of ambiguity. The worldwide community of pulmonary hypertension hopes that this classification will be more comprehensive and more useful to clinicians in the future. 
For the final presentation of the Plenary Session, professor Marius Hoeper from Germany took the stand. He focused on the evidence-based treatments of pulmonary hypertension, based on the current guidelines and the findings of clinical observations and discussions from physicians. Dr Hoeper discussed the importance of supportive therapies like diuretics, oxygen therapies and anticoagulants, and debated the issues of vaso-reactivity in pulmonary arterial hypertension and the criteria for respondents and non-respondents. He also stressed the importance of early diagnosis and its impact in the management of pulmonary hypertension. In addition, Dr Hoeper addressed the combination therapies. He presented some German data which showed that nearly 21% of the patients who have combination therapy are effectively on endothelin receptor and PDE5 inhibitors, whilst only 3% are on a combination of oral therapies including ERA or PDE5i and prostacyclin. 
The following session focused on updated issues in PH management and was chaired by Dr Yaseem Samman (KSA) and Dr Bassam Mahboub (UAE). The first talk was presented by Dr Qadar Pasha from India, from the Institute of Genomics and Integrative Biology in Delhi. He summarized in a very eloquent and clear manner the molecular aspects of pulmonary hypertension, specifically regarding the role of particular genomics in vascular functions and dysfunctions. He presented the recent gene findings and different aspects of recent chromosome genes and their chromosomal localization, and the reflection of function.
This was followed by a presentation from Dr Ghazwan Butrous from the University of Kent on whether post-capillary pulmonary hypertension should be treated with the available vasodilators. Dr Butrous defined both post-capillary pulmonary hypertension and the available so-called vasodilator treatment for pulmonary hypertension, and discussed its function in the treatment of post-capillary PH. He showed that early studies with prostacyclin and ERA were not encouraging due to various reasons. However, the experimental findings with PDE5i were more encouraging but there is no current evidence-based medicine, and the recent trial, which was mainly supported by the NIH, hasn't yet been reported. Therefore, the conclusion so far reads that an early experiment with small trials on pulmonary hypertension vasodilator therapy is encouraging, but the  evidence-based medicine larger clinical trials did not show any benefit of prostacyclin and ERA. We are still awaiting the results of PDE5i inhibitors. Based on this, Dr Butrous argued that in our current knowledge,  vasodilators are not considered a standard therapy for post capillary pulmonary hypertension. 
Dr Irene Lang from Austria followed this presentation by a talk on her experiences in her centre at the AKJ Vienna Hospital regarding the subcutaneous treprostinil. She discussed the value of using this method, as well as its difficulties and practical applications, and the importance of having a proper team that can help with the management of this form of modalities in the treatment of pulmonary hypertension patients. Attendees agreed that Dr Lang's presentation was a very enlightening experience with real life examples, providing a lot of practical implications as well as demonstrating the importance of new developments in the technology of delivering drugs. 
After a short lunch break, a new session focused on right ventricular pathophysiolgy secondary to pulmonary hypertension chaired by Dr Javid Khan and Manal Al Hazmi. The first presenter was Dr Paul Hassoun from John Hopkins Medical Centre. Dr Hassoun discussed the right ventricular response to pulmonary vascular load and he compared this to pulmonary hypertension syndrome: the IPAH versus scleroderma associated with pulmonary hypertension of the right ventricle, and described the hemodynamic serum markers and the cardiac factors that may impact on survival. He concluded that scleroderma related to pulmonary hypertension carries a grave prognosis, and several hemodynamic predictors survivors have been identified from their research group, and accordingly, dysfunction is a prominent factor in the prognosis of this condition. The possible mechanisms for this cardiac involvement could be not only the hemodynamic but neuro-hormonal myocardial dysfunction, which includes fibrosis inflammation and perfusion defect. For the time being no medical treatment has been established.

This was followed by Dr Abdulmajeed AlOtay from Saudi Arabia, who discussed the importance of using the new technology of the cardiac MRI in the assessment of right ventricular function and PA reactions in pulmonary hypertension. An interesting presentation, Dr AlOtay discussed, with a lot of convincing evidence, the importance of the use of the MRI in the diagnosis of pulmonary hypertension and management of patients with pulmonary hypertension.
The session on the right ventricle in PH was closed by Dr Marius Hoeper, who discussed the therapeutic intervention in right heart failure. In this presentation, Dr Hoeper discussed the importance of accurately identifying the role of the clinical condition of the right heart ventricle when managing a patient with pulmonary hypertension. In his conclusion, he showed that patients who have been treated aggressively with cardiac pumps also might benefit from a transplant which can have vital and successful results. Dr Hoeper showed encouraging examples of the use of venoarterial ECMO in these patients, via the positive response to the treatment. The attendees agreed that this clinical presentation in the management of severe right ventricle failure in patients with pulmonary hypertension was very illuminating.
During the last session, Dr. Saleh al Dammas presented a well balanced treatment of the COPD bronchodilators. Dr al Dammas stated that COPD is the fourth leading cause of death and indicated that the majority of these death happen in heavily polluted areas; for examples, 50% of these deaths were in China. Dr al Dammas noted that COPD in the Kingdom of Saudi Arabia is still understudied. This is worrying, as there are many risk factors prevalent in Saudi Arabia. Dr al Dammas and his group recently published small studies which found that 501 out of 1380 smokers were eligible for some form of analysis. Additionally, they found that 14% had FEV1 of less than 0.7. Almost 44% of these were on stage III COPD. This shows that trials with current PH specific agents like bosentan, sildenafil, iloprost and parentertal prostanoids and even volume reduction surgery were being used. His conclusion is that Pulmonary Hypertension is common for COPD patients, and a small percentage of COPD patients have severe pulmonary hypertension that behaves like idiopathic pulmonary arterial hypertension, This should be suspected when there is unexplained functional decline, thus right heart ventricle is a procedure of choice in the diagnosis of PH in patients with COPD. He stressed the importance of more research to evaluate the presence of pulmonary hypertension in groups of patients.
Dr Khalid Al Najashi followed with a presentation on the management of pregnant patients with pulmonary hypertension. He presented three cases on these issues and highlighted the importance of careful clinical management. He concluded that pulmonary hypertension, irrespective of the cause, is an absolute contraindication of pregnancy and once female pulmonary hypertension patients are of childbearing age, they should be counselled in a tertiary centre. If a woman with pulmonary hypertension becomes pregnant, this conceives a high alert emergency state whereby a multidisciplinary approach is needed which focuses on aggressive fluid management post-partum and initiation of vasodilators.
The last presentation of the day was from Dr Gerald Simmoneau who talked about PH registries: opportunities and difficulties, and focused on the information that may be derived from registries in pulmonary hypertension. He also related the experience of the French PAH registry, which is one of the biggest registries in the world involving 674 patients in 17 medical centers in France. The PAH registry published that idiopathic pulmonary hypertension is the predominant cause of pulmonary arterial hypertension with 39%, followed by connective tissue diseases (15%), congenital heart diseases (11%) and portal hypertension (10%). The majority of the patients (63%) were identified as Class III. In his presentation and the assessment of survival of PAH in the modern era, it became clear that despite our advances in treatment we still have not achieved our goal, as the majority of the mortality rates is associated with functional flaws. This indicates the importance of early diagnosis in this group of patients. Dr. Simonneau's summary reiterated that a pulmonary hypertension registry can provide important information in addition to RCTs. However, the methodology for these registries should be particularly rigorous as its subsequent data can generate new hypotheses that form the basis for prospective studies. The design is to be adapted to the questions one wants to answer. 

The meeting was closed and followed by a very pleasant gala dinner in one of the beach hotels in Dubai. The dinner was kicked off with a welcome speech from Dr Majdy Idrees who presented a token of appreciation to the guest speakers, the active members of the SAPH and the doctors who participated in enhancing the understanding of PH in the region. 
The following day the final set of meetings began with a presentation of Dr Ghazwan Butrous on pulmonary hypertension in Clinical Year in Review. Dr Butrous presented highlights from the important papers and the lessons we have learned. The presentation is attached here for download. Next, Dr M Omar Galal held a very interesting talk on the issues of writing a manuscript and the ethics regarding its proper execution. This was considered a very useful lesson, especially for the younger attendees.  Dr Enas Batubara presented her paper, titled 'a novel approach to the management of sub-massive pulmonary embolism', which focused on the targeting of the right ventricle in massive pulmonary hypertension. Dr Batubara's paper included a study which was conducted at the division of Pulmonary Medicine in Riyadh Military Hospital, under the supervision of Dr Majdy Idrees. The objective of the study was to direct their target of pulmonary vascular resistance rather than eliminate the obstruction, to improve cardiac output, and also to decrease the risk of bleeding. Their conclusion is that in sub-massive pulmonary embolism, thromboembolic therapy towards decreasing PVRI is effective in improving the heamodynamic management associated with this condition. That strategy might turn out to be the most effective approach for treating this condition and might erase the need for thromboembolic therapy. This is only the outcome of a small study and needs to be confirmed in a larger randomized placebo-controlled study.

This was followed by a presentation of Dr AlaEldin Ahmed who spoke about pulmonary hypertension patients treated with pulmonary tuberculosis. He presented a series of patients from studies done in Khartoum, whereby pulmonary hypertension was diagnosed by the rigorous criteria of Doppler-echo cardiography which showed that 14 patients were suffering from pulmonary tuberculosis and pulmonary hypertension. In his conclusion Pulmonary hypertension of different grades occurs even in  cured pulmonary TB  but all patients had an abnormal chest radiograph. They were all of a relatively young age mean age is 43 years. This is a small study which obviously needs further evaluation and more work in this area.
Dr Hanaa Banjar presented cases on paediatric pulmonary hypertension and discussed different aetiology. In particular, she is trying to implement the lessons and instruction she learned from the Panama PVRI meeting in February 2011 on the classification of paediatric pulmonary hypertension, and stressed the importance of learning from these cases for the practical aspects of the suggested aspects of the classification.
After the coffee break the focus was on 'Pharma-economic and End of Life Issues', which saw presentations by three participants: Dr Meshal Al Mutairi, Dr Maha al Dabbagh (KSA) and Dr Jameel Al Ata (KSA). Dr Al Mutairi, from Saudi Arabia, discussed the pharma-economic models of pulmonary hypertension, in particular those implemented in Saudi Arabia. There are a lot of good lessons to be learned, particularly in countries that want to establish their pulmonary hypertension service. This was followed by Dr Maha al Dabbagh with a very exciting talk about the importance of the ethical manner in which to approach patients newly diagnosed with pulmonary hypertension. She spoke of the importance of counselling and her own experience with patients, especially those of the younger generation, and a way to communicate with patients as well as parents and guardians. This was a very illuminating talk that Dr al Dabbagh promised to write down for the PVRI Review. The last talk was by Dr Jameel al Ata who also discussed the ethical issues around the treatment of pulmonary hypertension. He discussed his own experiences with patients in the kingdom of Saudi Arabia. He also spoke of the implications regarding failure to comply with the guideline, or unnecessarily overusing a drug, and other such issues. In a way, this represents the great awareness and subsequent critical analysis and awareness amongst physicians regarding the way other physicians treat patients with pulmonary hypertension, especially in specialist centres.
The session closed with two workshops; one focused on the haemodynamics in pulmonary hypertension, moderated by Paul Hassoun and Majdy Idrees, and a second group, which saw presentations by Ahmed Ibrahim and Omar Tamimi discussing the echocardiography in the management of PH.
Overall the meeting was very successful, and showed real advances over its previous incarnations. The attendees in particular were very engaged with the discussion and debate, and this was noticeable even during the coffee breaks and lunch periods. In general, this was considered a very successful meeting which has shown that the mission of the SAPH and PVRI is being achieved: both by the increased awareness of pulmonary hypertension worldwide and by the enhancement of the understanding of illness, and its treatment, at even the local level.
The 5th SAPH meeting is set for April 2012 and hopes to attract more PVRI members and Fellows, so the participation and debate between both organisations may continue to grow.