{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T18:55:23Z","timestamp":1793213723626,"version":"4.5.0"},"reference-count":88,"publisher":"Oxford University Press (OUP)","issue":"12","license":[{"start":{"date-parts":[[2021,6,1]],"date-time":"2021-06-01T00:00:00Z","timestamp":1622505600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":["www.atsjournals.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2021,6,15]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>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\u2019s 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-\u03b2 ligand trap pharmacotherapy, remote pulmonary arterial pressure monitoring, next-generation imaging using inert gas\u2013based 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.<\/jats:p>","DOI":"10.1164\/rccm.202012-4317so","type":"journal-article","created":{"date-parts":[[2021,4,16]],"date-time":"2021-04-16T15:05:39Z","timestamp":1618585539000},"page":"1472-1487","update-policy":"https:\/\/doi.org\/10.1164\/crossmarkpolicies","source":"Crossref","is-referenced-by-count":177,"title":["Pulmonary Arterial Hypertension: Diagnosis, Treatment, and Novel Advances"],"prefix":"10.1093","volume":"203","author":[{"given":"Bradley A.","family":"Maron","sequence":"first","affiliation":[{"name":"Division of Cardiovascular Medicine, Brigham and Women\u2019s Hospital and Harvard Medical School, Harvard University, Boston, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7292-2085","authenticated-orcid":false,"given":"Steven H.","family":"Abman","sequence":"additional","affiliation":[{"name":"Section of Pulmonary Medicine, Children\u2019s Hospital Colorado and the University of Colorado Anschutz Medical Center, University of Colorado, Aurora, Colorado"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C. Greg","family":"Elliott","sequence":"additional","affiliation":[{"name":"Intermountain Medical Center and the University of Utah, Salt Lake City, Utah"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert P.","family":"Frantz","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachel K.","family":"Hopper","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology, Department of Pediatrics, School of Medicine, Stanford University, Palo Alto, California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evelyn M.","family":"Horn","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine, Weill Cornell Graduate School of Medical Sciences, Cornell University, New York, New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark R.","family":"Nicolls","sequence":"additional","affiliation":[{"name":"Veterans Affairs Palo Alto Health Care System and School of Medicine, Stanford University, Stanford, California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oksana A.","family":"Shlobin","sequence":"additional","affiliation":[{"name":"Advanced Lung Disease and Transplant Program, Inova Fairfax Hospital, Falls Church, Virginia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sanjiv J.","family":"Shah","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine, Feinberg School of Medicine, Northwestern University, Chicago, Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabor","family":"Kovacs","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, Medical University of Graz and Ludwig Boltzmann Institute for Lung Vascular Research, Graz, Austria; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2834-7466","authenticated-orcid":false,"given":"Horst","family":"Olschewski","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, Medical University of Graz and Ludwig Boltzmann Institute for Lung Vascular Research, Graz, Austria; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erika B.","family":"Rosenzweig","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and"},{"name":"Department of Medicine, Columbia University Vagelos College of Physicians and Surgeons, New York, New York"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,6,15]]},"reference":[{"key":"2026030322071602000_B1","doi-asserted-by":"crossref","first-page":"1801913","DOI":"10.1183\/13993003.01913-2018","article-title":"Haemodynamic definitions and updated clinical classification of pulmonary hypertension","volume":"53","author":"Simonneau","year":"2019","journal-title":"Eur Respir J"},{"key":"2026030322071602000_B2","doi-asserted-by":"crossref","first-page":"1056","DOI":"10.1001\/jamacardio.2016.4471","article-title":"Diagnosis, treatment, and clinical management of pulmonary arterial hypertension in the contemporary era: a review","volume":"1","author":"Maron","year":"2016","journal-title":"JAMA Cardiol"},{"key":"2026030322071602000_B3","doi-asserted-by":"crossref","first-page":"1861","DOI":"10.1161\/CIRCULATIONAHA.118.037613","article-title":"A concerning trend for patients with pulmonary hypertension in the era of evidence-based medicine","volume":"139","author":"Maron","year":"2019","journal-title":"Circulation"},{"key":"2026030322071602000_B4","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1183\/09059180.00008211","article-title":"REVEAL: a contemporary US pulmonary arterial hypertension registry","volume":"21","author":"McGoon","year":"2012","journal-title":"Eur Respir Rev"},{"key":"2026030322071602000_B5","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1183\/09031936.00027613","article-title":"Sex and haemodynamics in pulmonary arterial hypertension","volume":"43","author":"Ventetuolo","year":"2014","journal-title":"Eur Respir J"},{"key":"2026030322071602000_B6","doi-asserted-by":"crossref","first-page":"e32","DOI":"10.1164\/rccm.201709-1821ST","article-title":"Health disparities in patients with pulmonary arterial hypertension: a blueprint for action. 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