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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

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
Unexplained syncope: clinical management.
Wolff GS
Pacing Clin Electrophysiol 1997 Aug;20(8 Pt 2):2043-7. doi: 10.1111/j.1540-8159.1997.tb03624.x. PMID: 9272505

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
Recognition of Sympathetic Crashing Acute Pulmonary Edema (SCAPE) and use of high-dose nitroglycerin infusion.
Paone S, Clarkson L, Sin B, Punnapuzha S
Am J Emerg Med 2018 Aug;36(8):1526.e5-1526.e7. Epub 2018 May 10 doi: 10.1016/j.ajem.2018.05.013. PMID: 29776826
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
Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial.
Simonneau G, Barst RJ, Galie N, Naeije R, Rich S, Bourge RC, Keogh A, Oudiz R, Frost A, Blackburn SD, Crow JW, Rubin LJ; Treprostinil Study Group
Am J Respir Crit Care Med 2002 Mar 15;165(6):800-4. doi: 10.1164/ajrccm.165.6.2106079. PMID: 11897647

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
HIV-related pulmonary hypertension.
Limsukon A, Saeed AI, Ramasamy V, Nalamati J, Dhuper S
Mt Sinai J Med 2006 Nov;73(7):1037-44. PMID: 17195895

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
Rehabilitation in patients with pulmonary arterial hypertension.
Keusch S, Turk A, Saxer S, Ehlken N, Grunig E, Ulrich S, On Behalf Of The Swiss Society Of Pulmonary Hypertension
Swiss Med Wkly 2017;147:w14462. Epub 2017 Jul 7 doi: 10.4414/smw.2017.14462. PMID: 28695558
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

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

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