Pulmonary arterial hypertension: Diagnosis, treatment, and novel advances

Bradley A. Maron*, Steven H. Abman, C. Greg Elliott, Robert P. Frantz, Rachel K. Hopper, Evelyn M. Horn, Mark R. Nicolls, Oksana A. Shlobin, Sanjiv J. Shah, Gabor Kovacs, Horst Olschewski, Erika B. Rosenzweig

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

50 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)1472-1487
Number of pages16
JournalAmerican journal of respiratory and critical care medicine
Issue number12
StatePublished - Jun 15 2021


  • Pulmonary arterial hypertension
  • Pulmonary hypertension
  • Risk stratification
  • Treatment

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine


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