Pulmonary Hypertension: From Pathophysiology to Clinical Management

Dear Colleagues,

Pulmonary hypertension (PH) affects up to 1% of the world's population and 10% of people over the age of 65. The classification of PH into five groups based on the underlying pathophysiology is particularly important due to its therapeutic implications. Only the rarer groups of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are usually treated specifically.

The scientific history of PH is composed of a number of remarkable innovations. Since the first autopsy description in 1891 and the introduction of right heart catheterisation in 1929, numerous pathophysiological, diagnostic and therapeutic advances have been made. Following epoprostenol, the first PH drug approved in 1995, many other specific PH treatments have been developed on the basis of detailed pathophysiological research in the last thirty years, including endothelin receptor antagonists, other prostacyclin analogues or receptor agonists, PDE5 inhibitors, sGC stimulators and, most recently, a unique activin signalling pathway inhibitor called sotatercept, which is currently delivering remarkable results. In addition, pulmonary endarterectomy has established itself as an effective, safe and potentially curative treatment option for operable CTEPH patients. More recently, balloon pulmonary angioplasty has also become established for inoperable CTEPH patients. Nevertheless, mortality among PH patients remains high; PH cannot be cured in most patients, and for certain PH groups, such as PH associated with left heart disease or PH associated with lung disease, there is still little evidence for specific treatments.

To further improve the pathophysiological understanding and treatment of PH, this Special Issue invites you to participate in this ongoing process. We look forward to receiving your submissions of original papers, review articles, and meta-analyses aimed at improving the pathophysiological and/or therapeutic knowledge about PH.

Dr. Dirk Bandorski
Dr. Lukas Ley
Guest Editors

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