Current Challenges in Pulmonary Hypertension

Pulmonary hypertension and its consequences on right heart function contribute markedly to the global burden of chronic pulmonary diseases. Since the 1950s and the development of right-heart catheterization techniques, amazing achievements have been made in pulmonary vascular medicine and five World Pulmonary Hypertension conferences have taken place in Geneva, Switzerland (1973); Evian, France (1998); Venice, Italy (2003); Dana Point, CA (2008), and Nice, France (2013). In parallel, important guidelines have provided a robust hemodynamic definition of pulmonary hypertension as an increase in mean pulmonary arterial pressure ≥ 25 mm Hg at rest, as assessed by right heart catheterization. In addition, a clinical classification has described five major subcategories of pulmonary hypertension including pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension that have been subject to the most rapid advancements in terms of knowledge and treatment options in past decades.

This fast-growing field of cardiorespiratory medicine is the topic of the present issue of Seminars in Respiratory and Critical Care Medicine, in which highly respected international authors have been invited to update our readers on major aspects of pulmonary hypertension. This issue opens with an article on pathology and pathobiology describing important aspects of pulmonary vascular remodeling and its mechanisms. It closes with an article on the complex double-edge topic of tyrosine kinase inhibition with possible pulmonary vascular benefits and significant risks in treated patients. Other topics include descriptions of idiopathic and heritable pulmonary arterial hypertension as well as specific topics on pulmonary arterial hypertension in the setting of connective tissue diseases and HIV infection. Other articles focus on the relatively common discussion of pulmonary hypertension complicating chronic respiratory diseases and chronic thromboembolic pulmonary disease. Pediatric forms of the disease are described in a specific article, as well as pulmonary hypertension in the elderly and the important discussion of left heart failure with preserved ejection fraction. Birth control and pregnancy are extensively analyzed in the present issue. Last but not the least, emergency care of right heart failure in pulmonary hypertensive patients and lung transplantation are addressed in articles that have important practical consequences for the care of these severe patients.

The burden of pulmonary vascular diseases remains certainly underestimated worldwide and requires much attention from the medical community. Better characterization and management of the subjects displaying pulmonary vascular conditions is of major importance to improve patients' outcomes. We hope that the following articles will contribute to better awareness, characterization, and care of patients presenting with pulmonary hypertension.