Introducing the Pulmonary Hypertension Thematic Series

Introducing the Pulmonary Hypertension Thematic SeriesIt has been nearly 50 years since the World Health Organization first recognized pulmonary hypertension (PH) as a specific disease (1).At that time, the diagnostic criteria, clinical framework, and therapeutic approach to PH were rudimentary by any standard, informed largely by descriptive reports from autopsy specimens emphasizing the dramatic lung plexogenic arteriopathy that is pathognomonic for pulmonary arterial hypertension (PAH) (2).However, a relatively small group of international investigators at the time were determined to muster focus on pulmonary circulatory disorders, beginning an intense and unrelenting narrative that has transformed PH from a universally fatal clinical entity to a contemporary disease characterized by an evidence-based definition (3), international registries that optimize phenotyping and prognostication (4, 5), and an expanding armamentarium of disease-specific therapeutics that now offer patients a measure of optimism ( 6).The arc of progress in PH continues along a steep trajectory, reaching novel heights that clarify genetic and other molecular disease drivers, leverage next-generation analytics for subphenotyping patients (7), emphasize early disease detection (8), and introduce sotatercept as the first novel drug class for patients with PAH in nearly two decades that is under consideration for U.S. Food and Drug Administration approval (9).Understanding this changing landscape can be challenging but is pertinent to general practitioners, PH specialists, and scientists alike, because diagnosis is often late or incorrect and inappropriate treatment is common (10), and continued progress in developing pharmacotherapeutics will most certainly hinge on maintaining a broad-based investment into basic and translational mechanisms of disease.To bring the readership up to date on these important topics, the Journal is introducing a new thematic series on PH.The contributions will include a comprehensive state-of-the-art summary of the basic, translational, and clinical concepts that define pulmonary vascular diseases today.This will detail the most up-to-date information on the approach to treatment and risk stratification, including emergent ideas on useful clinical trial endpoints, and discussion on sotatercept biology and clinical efficacy.Other key topics that will be covered in the series include focused consideration of the concept of disease modification in PH, which underscores the high bar set for defining successful pharmacotherapeutics in the next era, an update on postpulmonary embolism clinical phenotypes, fresh guidance on the management of patients with lung disease-PH, and new directions that further knowledge of sex as a biological variable in PH pathogenesis and clinical presentation.