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Nice 5th World Symposium on Pulmonary Hypertension Report

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Veronica Franco, MD
ISHLT PH Council Communications Liaison
The Ohio State University
Columbus, Ohio, USA

As a participant in the 5th World Symposium on Pulmonary Hypertension (WSPH), held February 2013 in Nice, France, I was struck by several things—first was the beautiful scenery. The best of Nice: breathing fresh Mediterranean air while walking along the Promenade des Anglais, viewing the deep blue waters of the Cote d'Azur and an eagle eye view of the whole city from atop Castle Hill, eating exquisite Gelato and French cuisine, and best just sitting at a café at Plaza Massena and relaxing. Nice is one of my favorite places to visit, thus, is not surprising the ISHLT board of directors choose this amazing city for their meeting in 2015.

The WSPH convenes every 5 years to discuss updates in the field. The symposium is designed to create guidelines to improve clinical practice and to standardize both pathologic and clinical definitions. A consensus on standard of care therapy is difficult in an orphan disease but these guidelines will help physicians better treat their patients. More importantly, it provides perspectives of future investigations. The 5th WSPH had a record attendance, in excess of 1000 participants—a diverse group that included clinicians, researchers, pharmacists and industry personnel all sitting in one room, the main auditorium.

Findings: The definition of PAH did not change, defined as a mean pulmonary artery pressure (mPAP) ≥ 25 mmHg and wedge pressure ≤ 15 mmHg. The cutoff of mPAP 25 mmHg was chosen as was the valued used in all clinical trials and previous PAH registries. Pulmonary vascular resistance (PVR) will not be included, as was in Dana Point [1]. A very interesting discussion took place about the true upper limits of normal for mPAP: ~ 20 mmHg based on published data. Nonetheless, patients with mPAP in the 20-24 range have not been included in clinical trials and more data regarding the natural history is needed. Based in current epidemiological data, it is not even clear if these patients will develop PAH in the future, but it is particularly concerning for the very high risk populations, such as patients with scleroderma.

Exercise-induced PH remains controversial. Abnormal exercise-induced pulmonary pressures could allow for an earlier diagnosis of PAH, but thus far, there is not conclusive evidence of a universal abnormal threshold in mPAP. Studies showed that in younger persons, < 50 yrs, mPAP ~ 35 mmHg can be considered normal during exercise [2]. More importantly, in contrast to pressures at rest, mPAP during exercise is largely age-dependent, presumably as a result of increasing stiffness of the left ventricle and the pulmonary vessels and mPAP ~ 30 mmHg during mild exercise have been seen in 50% of apparently healthy subjects aged > 50 yrs. The definition of a "normal" value for the wedge with exercise is also still debated. Without the appropriate data and consensus, no recommendation could be made on exercise hemodynamics.

The right ventricle (RV) continued to be an important topic at the symposium. Since our therapeutics have not cured the disease, it is clear that we need therapies that alter both the pulmonary vasculature and the RV. Our therapies help improve patients' exercise capacity and recently completed trials reaffirm that they improve morbidity (clinical worsening events) [3-5]. The message at the 5th WSPH was clear: we should aim for normal RV function by imaging (RV ejection fraction and size), biomarkers (natriuretic peptides), hemodynamics (right atrial pressure < 8 mmHg and cardiac index ≥ 2.5 L/mg/m2 and functional class (I or II)! The importance of objective evaluation of RV function in clinical trials will require study in early trial development so we can learn what is a clinically relevant improvement. Adaptive and creative trial designs are needed as well as a better way to utilize our limited patient cohort in trial development.

Finally, with the globalization of our pivotal trials the ethics and needs that now arise in performing these trials will need to be considered. The final guidelines manuscript should be published in the upcoming year. ISHLT San Diego will discuss many of these topics in more detail.

Disclosure Statement: The author has no conflicts of interest to report.


  1. Galie N, Hoeper MM, Humbert M, et al. Guidelines on the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2009; 34: 1219-1263.
  2. Kovacs G, Berghold A, Scheidl S,et al. Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J 2009; 34: 888-894.
  3. Gomberg-Maitland M, Dufton C, Oudiz R, et al. Compelling evidence of long-term outcomes in pulmonary arterial hypertension? J Am Coll Cardiol 2011;57:1053-1061
  4. Ghofrani HA, Galiè N, Grimminger F, et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med 2013; 369: 330-340.
  5. Pulido T, Adzerikho I, Channick RN, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369: 809-818.

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