Pulmonary Hypertension: Mechanisms and Management, History and Future

In the 1950s, Donald Heath put forward the first description of the pathology of “hypertensive pulmonary vascular disease”, highlighting distinct muscularisation of small pulmonary arteries and severe intimal fibrosis that almost totally occluded the vessels. At that time, the prognosis for patients with pulmonary arterial hypertension was very poor and there were few treatment options. Remarkable advances in the pharmacological management of pulmonary hypertension have been achieved in the past 20 years but the search continues for treatments to reverse the relentless vascular remodelling that characterises the disease. With expanding research tools and big data, systems biology approaches allow the identification of pathways and genes that are commonly, and uniquely active, at different stages of disease progression and treatment. Finding druggable targets among these pathways that translate from pre-clinical models of disease to human treatments has been challenging. However, through detailed assessments using imaging and biomarkers, a better understanding of different clinical phenotypes and risk will develop, and should allow stratification of patients into cohorts that may respond to specific therapeutics. In this Research Topic in Frontiers in Medicine, we aim to provide a broad overview of novel mechanisms and disease drivers in pulmonary hypertension, an outline of methods used in deep phenotyping of patients, and a focus on potential therapeutic strategies.

In the UK, Sheffield has a long and proud history in pulmonary hypertension dating back over 60 years. Donald Heath was a junior doctor in Sheffield at the time he and Whitaker published their histological description of pulmonary hypertension. In the more recent past, the clinical service in Sheffield has expanded to become one of the largest in Europe. To strengthen links between basic science, clinical and imaging research, a Donald Heath research programme has recently been established in Sheffield, and this Research Topic will feature several reviews and opinion articles from international groups attending the launch symposium. The Topic will also be open to submissions of original research that compliment the Topic. The running theme will be one of personalised medicine and the use of genetic, proteomic and imaging metrics to classify patients and provide detailed insights into both pathogenesis and management of pulmonary hypertension.

We are grateful for the support of Actelion – a sponsor of this Research Topic – whose cooperation has contributed to fostering scientific discovery by reducing article publishing costs for some authors. We hereby state publicly that Actelion has had no editorial input in articles included in this research topic, thus ensuring that all aspects of this Research Topic were evaluated objectively, unbiased by any specific policy or opinion of Actelion.