Evolving Concepts in Pulmonary Hypertension

Marc Humbert, MD, PhD

Pulmonary hypertension is becoming an important global health problem that may complicate a wide range of conditions.[1] [2] [3] All age groups can be affected, and left heart and lung diseases are the leading causes of pulmonary hypertension worldwide.[1] [2] [3] In developing countries, pulmonary hypertension may also be due to highly prevalent infectious diseases, such as schistosomiasis.[1] Independently of its causes, pulmonary hypertension is associated with clinical deterioration and increased mortality.[1] [2] [3]

In the last 30 years, the pulmonary hypertension community has been very productive and innovative. A couple of years ago, the European Society of Cardiology/European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension summarized the currently available evidence.[2] [3] At the end of 2018, the proceedings of the 6th World Symposium on Pulmonary Hypertension should be published. In the interim, we believed that it was time to invite a group of international experts to discuss evolving concepts in pulmonary hypertension in a dedicated issue of Seminars in Respiratory and Critical Care Medicine.

Among others, recent developments discussed in this issue include initial combination therapy in pulmonary arterial hypertension,[2] [3] [4] [5] [6] and a variety of medical, surgical, and interventional approaches now available for chronic thromboembolic pulmonary hypertension.[7] [8] [9] [10] Finally, one should always consider the appropriate timing of lung transplantation, a life-saving option for eligible patients refractory to best standard of care.[11]

• References

• 1 Hoeper MM, Humbert M, Souza R. , et al. A global view of pulmonary hypertension. Lancet Respir Med 2016; 4 (04) 306-322
• 2 Galiè N, Humbert M, Vachiery JL. , et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 2015; 46 (04) 903-975
• 3 Galiè N, Humbert M, Vachiery JL. , et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2016; 37 (01) 67-119
• 4 Sitbon O, Jaïs X, Savale L. , et al. Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study. Eur Respir J 2014; 43 (06) 1691-1697
• 5 Galiè N, Barberà JA, Frost AE. , et al; AMBITION Investigators. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. N Engl J Med 2015; 373 (09) 834-844
• 6 Sitbon O, Sattler C, Bertoletti L. , et al. Initial dual oral combination therapy in pulmonary arterial hypertension. Eur Respir J 2016; 47 (06) 1727-1736
• 7 O'Connell C, Montani D, Savale L. , et al. Chronic thromboembolic pulmonary hypertension. Presse Med 2015; 44 (12 Pt 2): e409-e416
• 8 Simonneau G, D'Armini AM, Ghofrani HA. , et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2). Eur Respir J 2015; 45 (05) 1293-1302
• 9 Lang I, Meyer BC, Ogo T. , et al. Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension. Eur Respir Rev 2017; 26 (143) 160119
• 10 Matsubara H, Ogawa A. A long way to go after the initial experience with balloon pulmonary angioplasty. Eur Respir J 2017; 49 (06) 1700718
• 11 Savale L, Le Pavec J, Mercier O. , et al. Impact of high-priority allocation on lung and heart-lung transplantation for pulmonary hypertension. Ann Thorac Surg 2017; 104 (02) 404-411