Pulmonary Arterial Hypertension: Diagnosis, Treatment, and Novel Advances

• Published in: American journal of respiratory and critical care medicine Vol. 203; no. 12; pp. 1472 - 1487
• Main Authors: Maron, Bradley A, Abman, Steven H, Elliott, C Greg, Frantz, Robert P, Hopper, Rachel K, Horn, Evelyn M, Nicolls, Mark R, Shlobin, Oksana A, Shah, Sanjiv J, Kovacs, Gabor, Olschewski, Horst, Rosenzweig, Erika B
• Format: Journal Article
• Language: English
• Published: United States American Thoracic Society 15.06.2021
• Subjects: Adult; Aged; Aged, 80 and over; Cardiac Catheterization - methods; Cardiac Surgical Procedures - methods; Drug therapy; Early Diagnosis; Female; Humans; Male; Medical diagnosis; Medical treatment; Middle Aged; Pulmonary Arterial Hypertension - diagnosis; Pulmonary Arterial Hypertension - physiopathology; Pulmonary Arterial Hypertension - therapy; Pulmonary arteries; Pulmonary hypertension; Risk Factors; State-of-the-Art; Therapies, Investigational - methods; treatment; risk stratification; pulmonary arterial hypertension; pulmonary hypertension
• ISSN: 1073-449X; 1535-4970
• DOI: 10.1164/rccm.202012-4317SO

The diagnosis and management of pulmonary arterial hypertension (PAH) includes several advances, such as a broader recognition of extrapulmonary vascular organ system involvement, validated point-of-care clinical assessment tools, and focus on the early initiation of multiple pharmacotherapeutics in appropriate patients. Indeed, a principal goal in PAH today is an early diagnosis for prompt initiation of treatment to achieve a minimal symptom burden; optimize the patient's biochemical, hemodynamic, and functional profile; and limit adverse events. To accomplish this end, clinicians must be familiar with novel risk factors and the revised hemodynamic definition for PAH. Fresh insights into the role of developmental biology (i.e., perinatal health) may also be useful for predicting incident PAH in early adulthood. Emergent or underused approaches to PAH management include a novel TGF-β ligand trap pharmacotherapy, remote pulmonary arterial pressure monitoring, next-generation imaging using inert gas-based magnetic resonance and other technologies, right atrial pacing, and pulmonary arterial denervation. These and other PAH state of the art advances are summarized here for the wider pulmonary medicine community.