U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Syncope due to Pulmonary Hypertension

MedGen UID:
1710535
•Concept ID:
C5238880
•
Sign or Symptom

Definition

A condition in which syncope occurs associated with a sudden and severe increase in pulmonary vascular resistance. (ACC-AHA) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSyncope due to Pulmonary Hypertension

Professional guidelines

PubMed

Riociguat treatment in patients with chronic thromboembolic pulmonary hypertension: Final safety data from the EXPERT registry.
Ghofrani HA, Gomez Sanchez MA, Humbert M, Pittrow D, Simonneau G, Gall H, Grünig E, Klose H, Halank M, Langleben D, Snijder RJ, Escribano Subias P, Mielniczuk LM, Lange TJ, Vachiéry JL, Wirtz H, Helmersen DS, Tsangaris I, Barberá JA, Pepke-Zaba J, Boonstra A, Rosenkranz S, Ulrich S, Steringer-Mascherbauer R, Delcroix M, Jansa P, Šimková I, Giannakoulas G, Klotsche J, Williams E, Meier C, Hoeper MM; NEW COLLABORATORS LIST
Respir Med 2021 Mar;178:106220. Epub 2020 Nov 12 doi: 10.1016/j.rmed.2020.106220. PMID: 33540340
Pulmonary arterial hypertension: pathogenesis and clinical management.
Thenappan T, Ormiston ML, Ryan JJ, Archer SL
BMJ 2018 Mar 14;360:j5492. doi: 10.1136/bmj.j5492. PMID: 29540357Free PMC Article
Medical management of primary pulmonary hypertension.
McLaughlin VV
Expert Opin Pharmacother 2002 Feb;3(2):159-65. doi: 10.1517/14656566.3.2.159. PMID: 11829729

Recent clinical studies

Etiology

Balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (RACE): a multicentre, phase 3, open-label, randomised controlled trial and ancillary follow-up study.
Jaïs X, Brenot P, Bouvaist H, Jevnikar M, Canuet M, Chabanne C, Chaouat A, Cottin V, De Groote P, Favrolt N, Horeau-Langlard D, Magro P, Savale L, Prévot G, Renard S, Sitbon O, Parent F, Trésorier R, Tromeur C, Piedvache C, Grimaldi L, Fadel E, Montani D, Humbert M, Simonneau G
Lancet Respir Med 2022 Oct;10(10):961-971. Epub 2022 Aug 1 doi: 10.1016/S2213-2600(22)00214-4. PMID: 35926542
Pulmonary arterial hypertension: pathogenesis and clinical management.
Thenappan T, Ormiston ML, Ryan JJ, Archer SL
BMJ 2018 Mar 14;360:j5492. doi: 10.1136/bmj.j5492. PMID: 29540357Free PMC Article
Acute aortic dissection: pathogenesis, risk factors and diagnosis.
Gawinecka J, Schönrath F, von Eckardstein A
Swiss Med Wkly 2017;147:w14489. Epub 2017 Aug 25 doi: 10.4414/smw.2017.14489. PMID: 28871571
Riociguat for the treatment of pulmonary arterial hypertension.
Ghofrani HA, Galiè N, Grimminger F, Grünig E, Humbert M, Jing ZC, Keogh AM, Langleben D, Kilama MO, Fritsch A, Neuser D, Rubin LJ; PATENT-1 Study Group
N Engl J Med 2013 Jul 25;369(4):330-40. doi: 10.1056/NEJMoa1209655. PMID: 23883378
Riociguat for the treatment of chronic thromboembolic pulmonary hypertension.
Ghofrani HA, D'Armini AM, Grimminger F, Hoeper MM, Jansa P, Kim NH, Mayer E, Simonneau G, Wilkins MR, Fritsch A, Neuser D, Weimann G, Wang C; CHEST-1 Study Group
N Engl J Med 2013 Jul 25;369(4):319-29. doi: 10.1056/NEJMoa1209657. PMID: 23883377

Diagnosis

Medical Emergencies in Pulmonary Hypertension.
Seitler S, Dimopoulos K, Ernst S, Price LC
Semin Respir Crit Care Med 2023 Dec;44(6):777-796. Epub 2023 Aug 18 doi: 10.1055/s-0043-1770120. PMID: 37595615
Balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (RACE): a multicentre, phase 3, open-label, randomised controlled trial and ancillary follow-up study.
Jaïs X, Brenot P, Bouvaist H, Jevnikar M, Canuet M, Chabanne C, Chaouat A, Cottin V, De Groote P, Favrolt N, Horeau-Langlard D, Magro P, Savale L, Prévot G, Renard S, Sitbon O, Parent F, Trésorier R, Tromeur C, Piedvache C, Grimaldi L, Fadel E, Montani D, Humbert M, Simonneau G
Lancet Respir Med 2022 Oct;10(10):961-971. Epub 2022 Aug 1 doi: 10.1016/S2213-2600(22)00214-4. PMID: 35926542
Clinical features and survival in Takayasu's arteritis-associated pulmonary hypertension: a nationwide study.
Jiang X, Zhu YJ, Zhou YP, Peng FH, Wang L, Ma W, Cao YS, Pan X, Zhang GC, Zhang F, Fan FL, Wu BX, Huang W, Yang ZW, Hong C, Li MT, Wang YN, Xu XQ, Wang DL, Zhang SY, Jing ZC
Eur Heart J 2021 Nov 7;42(42):4298-4305. doi: 10.1093/eurheartj/ehab599. PMID: 34506618
Pulmonary arterial hypertension: pathogenesis and clinical management.
Thenappan T, Ormiston ML, Ryan JJ, Archer SL
BMJ 2018 Mar 14;360:j5492. doi: 10.1136/bmj.j5492. PMID: 29540357Free PMC Article
Acute aortic dissection: pathogenesis, risk factors and diagnosis.
Gawinecka J, Schönrath F, von Eckardstein A
Swiss Med Wkly 2017;147:w14489. Epub 2017 Aug 25 doi: 10.4414/smw.2017.14489. PMID: 28871571

Therapy

Balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (RACE): a multicentre, phase 3, open-label, randomised controlled trial and ancillary follow-up study.
Jaïs X, Brenot P, Bouvaist H, Jevnikar M, Canuet M, Chabanne C, Chaouat A, Cottin V, De Groote P, Favrolt N, Horeau-Langlard D, Magro P, Savale L, Prévot G, Renard S, Sitbon O, Parent F, Trésorier R, Tromeur C, Piedvache C, Grimaldi L, Fadel E, Montani D, Humbert M, Simonneau G
Lancet Respir Med 2022 Oct;10(10):961-971. Epub 2022 Aug 1 doi: 10.1016/S2213-2600(22)00214-4. PMID: 35926542
Pediatric pulmonary arterial hypertension.
Wu DC, Zhang HD, Jing ZC
Curr Hypertens Rep 2013 Dec;15(6):606-13. doi: 10.1007/s11906-013-0399-3. PMID: 24163011
Riociguat for the treatment of pulmonary arterial hypertension.
Ghofrani HA, Galiè N, Grimminger F, Grünig E, Humbert M, Jing ZC, Keogh AM, Langleben D, Kilama MO, Fritsch A, Neuser D, Rubin LJ; PATENT-1 Study Group
N Engl J Med 2013 Jul 25;369(4):330-40. doi: 10.1056/NEJMoa1209655. PMID: 23883378
Riociguat for the treatment of chronic thromboembolic pulmonary hypertension.
Ghofrani HA, D'Armini AM, Grimminger F, Hoeper MM, Jansa P, Kim NH, Mayer E, Simonneau G, Wilkins MR, Fritsch A, Neuser D, Weimann G, Wang C; CHEST-1 Study Group
N Engl J Med 2013 Jul 25;369(4):319-29. doi: 10.1056/NEJMoa1209657. PMID: 23883377
Bosentan.
Prakash A, Perry CM
Am J Cardiovasc Drugs 2002;2(5):335-43; discussion 343. doi: 10.2165/00129784-200202050-00006. PMID: 14727963

Prognosis

Balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (RACE): a multicentre, phase 3, open-label, randomised controlled trial and ancillary follow-up study.
Jaïs X, Brenot P, Bouvaist H, Jevnikar M, Canuet M, Chabanne C, Chaouat A, Cottin V, De Groote P, Favrolt N, Horeau-Langlard D, Magro P, Savale L, Prévot G, Renard S, Sitbon O, Parent F, Trésorier R, Tromeur C, Piedvache C, Grimaldi L, Fadel E, Montani D, Humbert M, Simonneau G
Lancet Respir Med 2022 Oct;10(10):961-971. Epub 2022 Aug 1 doi: 10.1016/S2213-2600(22)00214-4. PMID: 35926542
Clinical features and survival in Takayasu's arteritis-associated pulmonary hypertension: a nationwide study.
Jiang X, Zhu YJ, Zhou YP, Peng FH, Wang L, Ma W, Cao YS, Pan X, Zhang GC, Zhang F, Fan FL, Wu BX, Huang W, Yang ZW, Hong C, Li MT, Wang YN, Xu XQ, Wang DL, Zhang SY, Jing ZC
Eur Heart J 2021 Nov 7;42(42):4298-4305. doi: 10.1093/eurheartj/ehab599. PMID: 34506618
Molecular genetic framework underlying pulmonary arterial hypertension.
Southgate L, Machado RD, Gräf S, Morrell NW
Nat Rev Cardiol 2020 Feb;17(2):85-95. Epub 2019 Aug 12 doi: 10.1038/s41569-019-0242-x. PMID: 31406341
Pediatric pulmonary arterial hypertension.
Wu DC, Zhang HD, Jing ZC
Curr Hypertens Rep 2013 Dec;15(6):606-13. doi: 10.1007/s11906-013-0399-3. PMID: 24163011
Primary pulmonary hypertension.
Rashid A, Lehrman S, Romano P, Frishman W, Dobkin J, Reichel J
Heart Dis 2000 Nov-Dec;2(6):422-30. PMID: 11728293

Clinical prediction guides

Balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (RACE): a multicentre, phase 3, open-label, randomised controlled trial and ancillary follow-up study.
Jaïs X, Brenot P, Bouvaist H, Jevnikar M, Canuet M, Chabanne C, Chaouat A, Cottin V, De Groote P, Favrolt N, Horeau-Langlard D, Magro P, Savale L, Prévot G, Renard S, Sitbon O, Parent F, Trésorier R, Tromeur C, Piedvache C, Grimaldi L, Fadel E, Montani D, Humbert M, Simonneau G
Lancet Respir Med 2022 Oct;10(10):961-971. Epub 2022 Aug 1 doi: 10.1016/S2213-2600(22)00214-4. PMID: 35926542
Clinical features and survival in Takayasu's arteritis-associated pulmonary hypertension: a nationwide study.
Jiang X, Zhu YJ, Zhou YP, Peng FH, Wang L, Ma W, Cao YS, Pan X, Zhang GC, Zhang F, Fan FL, Wu BX, Huang W, Yang ZW, Hong C, Li MT, Wang YN, Xu XQ, Wang DL, Zhang SY, Jing ZC
Eur Heart J 2021 Nov 7;42(42):4298-4305. doi: 10.1093/eurheartj/ehab599. PMID: 34506618
Riociguat for the treatment of pulmonary arterial hypertension.
Ghofrani HA, Galiè N, Grimminger F, Grünig E, Humbert M, Jing ZC, Keogh AM, Langleben D, Kilama MO, Fritsch A, Neuser D, Rubin LJ; PATENT-1 Study Group
N Engl J Med 2013 Jul 25;369(4):330-40. doi: 10.1056/NEJMoa1209655. PMID: 23883378
Riociguat for the treatment of chronic thromboembolic pulmonary hypertension.
Ghofrani HA, D'Armini AM, Grimminger F, Hoeper MM, Jansa P, Kim NH, Mayer E, Simonneau G, Wilkins MR, Fritsch A, Neuser D, Weimann G, Wang C; CHEST-1 Study Group
N Engl J Med 2013 Jul 25;369(4):319-29. doi: 10.1056/NEJMoa1209657. PMID: 23883377
Pulmonary arterial hypertension: a comparison between children and adults.
Barst RJ, Ertel SI, Beghetti M, Ivy DD
Eur Respir J 2011 Mar;37(3):665-77. doi: 10.1183/09031936.00056110. PMID: 21357924Free PMC Article

Recent systematic reviews

Sex-Based Differences in the Presentation and Outcomes of Acute Pulmonary Embolism: A Systematic Review and Meta-Analysis.
Zhang Y, Qiu Y, Luo J, Zhang J, Yan Q
Tex Heart Inst J 2023 Aug 11;50(4) doi: 10.14503/THIJ-23-8113. PMID: 37577766Free PMC Article
Misdiagnosis of aortic dissection: A systematic review of the literature.
Lovatt S, Wong CW, Schwarz K, Borovac JA, Lo T, Gunning M, Phan T, Patwala A, Barker D, Mallen CD, Kwok CS
Am J Emerg Med 2022 Mar;53:16-22. Epub 2021 Dec 4 doi: 10.1016/j.ajem.2021.11.047. PMID: 34968970
Pulmonary vascular involvement of IgG4-related disease: Case series with a PRISMA-compliant systemic review.
Zhou Y, Shao L, Ruan W, Jin J, Xu H, Ying K, Wu X
Medicine (Baltimore) 2019 Feb;98(6):e14437. doi: 10.1097/MD.0000000000014437. PMID: 30732204Free PMC Article

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...