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Pulmonary Vascular Disorder

MedGen UID:
208897
•Concept ID:
C0854416
•
Disease or Syndrome

Definition

A category of non-neoplastic disorders that affect the blood vessels in the lungs. Representative examples include pulmonary embolism, pulmonary hypertension, and arteriovenous malformations. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPulmonary Vascular Disorder

Professional guidelines

PubMed

The management pattern and outcomes of chronic thromboembolic pulmonary hypertension: rationale and design for a Chinese real-world study.
Zhang S, Li Y, Yang Y, Gong S, Yang Z, Hong C, Cui X, Wan J, Ji Y, Chen H, Zhu L, Li C, Cheng Z, Zhang Y, Wang Q, Luo Q, Guo L, Ma G, Han B, Liu Z, Xiong C, Wang L, Ji Q, Li F, Wang S, Zhang Z, Zhang Y, Wang D, Gao Q, Huang Q, Xie W, Zhai Z, Wang C
BMC Pulm Med 2024 Jun 3;24(1):265. doi: 10.1186/s12890-024-03042-5. PMID: 38825688Free PMC Article
Emerging biologics for the treatment of pulmonary arterial hypertension.
Hye T, Hossain MR, Saha D, Foyez T, Ahsan F
J Drug Target 2023 Jun;31(5):1-15. Epub 2023 Apr 26 doi: 10.1080/1061186X.2023.2199351. PMID: 37026714Free PMC Article
Chronic Thromboembolic Pulmonary Hypertension: Epidemiology, Diagnosis, and Management.
Yandrapalli S, Tariq S, Kumar J, Aronow WS, Malekan R, Frishman WH, Lanier GM
Cardiol Rev 2018 Mar/Apr;26(2):62-72. doi: 10.1097/CRD.0000000000000164. PMID: 28832374

Recent clinical studies

Etiology

Myeloid-derived suppressor cells and pulmonary hypertension.
Zhang H, Li QW, Li YY, Tang X, Gu L, Liu HM
Front Immunol 2023;14:1189195. Epub 2023 Jun 7 doi: 10.3389/fimmu.2023.1189195. PMID: 37350962Free PMC Article
Low Pulmonary Artery Pulsatility Index by Echocardiography Is Associated With Increased Mortality in Pulmonary Hypertension.
Kane CJ, Salama AA, Pislaru C, Kane GC, Pislaru SV, Lin G
J Am Soc Echocardiogr 2023 Feb;36(2):189-195. Epub 2022 Sep 17 doi: 10.1016/j.echo.2022.09.003. PMID: 36126823
Chronic Thromboembolic Pulmonary Hypertension: Epidemiology, Diagnosis, and Management.
Yandrapalli S, Tariq S, Kumar J, Aronow WS, Malekan R, Frishman WH, Lanier GM
Cardiol Rev 2018 Mar/Apr;26(2):62-72. doi: 10.1097/CRD.0000000000000164. PMID: 28832374
Hepatopulmonary syndrome: favorable outcomes in the MELD exception era.
Iyer VN, Swanson KL, Cartin-Ceba R, Dierkhising RA, Rosen CB, Heimbach JK, Wiesner RH, Krowka MJ
Hepatology 2013 Jun;57(6):2427-35. Epub 2013 Feb 15 doi: 10.1002/hep.26070. PMID: 22996424
Managing chronic thromboembolic pulmonary hypertension: pharmacological treatment options.
Lang IM
Eur Respir Rev 2009 Mar;18(111):24-8. doi: 10.1183/09059180.00011110. PMID: 20956119

Diagnosis

The management pattern and outcomes of chronic thromboembolic pulmonary hypertension: rationale and design for a Chinese real-world study.
Zhang S, Li Y, Yang Y, Gong S, Yang Z, Hong C, Cui X, Wan J, Ji Y, Chen H, Zhu L, Li C, Cheng Z, Zhang Y, Wang Q, Luo Q, Guo L, Ma G, Han B, Liu Z, Xiong C, Wang L, Ji Q, Li F, Wang S, Zhang Z, Zhang Y, Wang D, Gao Q, Huang Q, Xie W, Zhai Z, Wang C
BMC Pulm Med 2024 Jun 3;24(1):265. doi: 10.1186/s12890-024-03042-5. PMID: 38825688Free PMC Article
Hepatopulmonary Disorders: Gas Exchange and Vascular Manifestations in Chronic Liver Disease.
Rodríguez-Roisin R, Krowka MJ, Agustí A
Compr Physiol 2018 Mar 25;8(2):711-729. doi: 10.1002/cphy.c170020. PMID: 29687908
Chronic Thromboembolic Pulmonary Hypertension: Epidemiology, Diagnosis, and Management.
Yandrapalli S, Tariq S, Kumar J, Aronow WS, Malekan R, Frishman WH, Lanier GM
Cardiol Rev 2018 Mar/Apr;26(2):62-72. doi: 10.1097/CRD.0000000000000164. PMID: 28832374
Lung involvement in systemic sclerosis.
Hassoun PM
Presse Med 2011 Jan;40(1 Pt 2):e3-e17. Epub 2010 Dec 30 doi: 10.1016/j.lpm.2010.08.006. PMID: 21195581Free PMC Article
Chronic thromboembolic pulmonary hypertension: an updated review.
Lang IM, Klepetko W
Curr Opin Cardiol 2008 Nov;23(6):555-9. doi: 10.1097/HCO.0b013e328311f254. PMID: 18830069

Therapy

Emerging biologics for the treatment of pulmonary arterial hypertension.
Hye T, Hossain MR, Saha D, Foyez T, Ahsan F
J Drug Target 2023 Jun;31(5):1-15. Epub 2023 Apr 26 doi: 10.1080/1061186X.2023.2199351. PMID: 37026714Free PMC Article
Chronic Thromboembolic Pulmonary Hypertension: Epidemiology, Diagnosis, and Management.
Yandrapalli S, Tariq S, Kumar J, Aronow WS, Malekan R, Frishman WH, Lanier GM
Cardiol Rev 2018 Mar/Apr;26(2):62-72. doi: 10.1097/CRD.0000000000000164. PMID: 28832374
Managing chronic thromboembolic pulmonary hypertension: pharmacological treatment options.
Lang IM
Eur Respir Rev 2009 Mar;18(111):24-8. doi: 10.1183/09059180.00011110. PMID: 20956119
Clinical relevance of ventilation-perfusion inequality determined by inert gas elimination.
Rodriguez-Roisin R, Wagner PD
Eur Respir J 1990 Apr;3(4):469-82. PMID: 2163880

Prognosis

Allosteric site identification, virtual screening and discovery of a sulfonamide Hsp110-STAT3 interaction inhibitor for the treatment of hypoxic pulmonary arterial hypertension.
Zhao C, Wu Y, Li M, Tan W, Hu Y, Wang Y, Gao R, Hu L, Li Q
Eur J Med Chem 2024 Dec 5;279:116855. Epub 2024 Sep 7 doi: 10.1016/j.ejmech.2024.116855. PMID: 39260318
Pulmonary Complications of Portal Hypertension.
Bommena S, Fallon MB
Clin Liver Dis 2024 Aug;28(3):467-482. Epub 2024 May 1 doi: 10.1016/j.cld.2024.03.005. PMID: 38945638
The management pattern and outcomes of chronic thromboembolic pulmonary hypertension: rationale and design for a Chinese real-world study.
Zhang S, Li Y, Yang Y, Gong S, Yang Z, Hong C, Cui X, Wan J, Ji Y, Chen H, Zhu L, Li C, Cheng Z, Zhang Y, Wang Q, Luo Q, Guo L, Ma G, Han B, Liu Z, Xiong C, Wang L, Ji Q, Li F, Wang S, Zhang Z, Zhang Y, Wang D, Gao Q, Huang Q, Xie W, Zhai Z, Wang C
BMC Pulm Med 2024 Jun 3;24(1):265. doi: 10.1186/s12890-024-03042-5. PMID: 38825688Free PMC Article
Myeloid-derived suppressor cells and pulmonary hypertension.
Zhang H, Li QW, Li YY, Tang X, Gu L, Liu HM
Front Immunol 2023;14:1189195. Epub 2023 Jun 7 doi: 10.3389/fimmu.2023.1189195. PMID: 37350962Free PMC Article
Hepatopulmonary syndrome: favorable outcomes in the MELD exception era.
Iyer VN, Swanson KL, Cartin-Ceba R, Dierkhising RA, Rosen CB, Heimbach JK, Wiesner RH, Krowka MJ
Hepatology 2013 Jun;57(6):2427-35. Epub 2013 Feb 15 doi: 10.1002/hep.26070. PMID: 22996424

Clinical prediction guides

Low Pulmonary Artery Pulsatility Index by Echocardiography Is Associated With Increased Mortality in Pulmonary Hypertension.
Kane CJ, Salama AA, Pislaru C, Kane GC, Pislaru SV, Lin G
J Am Soc Echocardiogr 2023 Feb;36(2):189-195. Epub 2022 Sep 17 doi: 10.1016/j.echo.2022.09.003. PMID: 36126823
Mammalian target of rapamycin overexpression antagonizes chronic hypoxia-triggered pulmonary arterial hypertension via the autophagic pathway.
Li L, Wang X, Wang L, Qu L, Zhu X, Li M, Dang X, Li P, Gao Y, Peng Z, Pan L, Wan L
Int J Mol Med 2015 Jul;36(1):316-22. Epub 2015 May 26 doi: 10.3892/ijmm.2015.2224. PMID: 26017061
Glycogen synthase kinase 3beta contributes to proliferation of arterial smooth muscle cells in pulmonary hypertension.
Sklepkiewicz P, Schermuly RT, Tian X, Ghofrani HA, Weissmann N, Sedding D, Kashour T, Seeger W, Grimminger F, Pullamsetti SS
PLoS One 2011 Apr 18;6(4):e18883. doi: 10.1371/journal.pone.0018883. PMID: 21533110Free PMC Article
Managing chronic thromboembolic pulmonary hypertension: pharmacological treatment options.
Lang IM
Eur Respir Rev 2009 Mar;18(111):24-8. doi: 10.1183/09059180.00011110. PMID: 20956119
The importance of predicting the prognosis in patients with hepatopulmonary syndrome: a simple scoring system.
Bottari G, Mazzeo AT, Santamaria LB
Transplant Proc 2006 Apr;38(3):795-7. doi: 10.1016/j.transproceed.2006.01.036. PMID: 16647473

Recent systematic reviews

Appraisal and evaluation of the quality of life in pulmonary arterial hypertension instruments: A systematic review using COSMIN methodology.
Irene G, Nadia C, Marzia L, Iovino P, Ercole V
Respir Med 2024 Nov-Dec;234:107829. Epub 2024 Oct 6 doi: 10.1016/j.rmed.2024.107829. PMID: 39378916

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