U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Chronic thromboembolic pulmonary hypertension

MedGen UID:
440560
•Concept ID:
C2748504
•
Finding
Synonyms: CTEPH, DVT-NEGATIVE, SUSCEPTIBILITY TO; Pulmonary hypertension, chronic thromboembolic, without deep vein thrombosis, susceptibility to
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0013024
OMIM®: 612862
Orphanet: ORPHA70591

Definition

A rare complication of acute pulmonary embolism (PE), either symptomatic or not, that is characterized by fibrotic intravascular material occlusion of pulmonary arteries in combination with a secondary microvasculopathy of vessels less than 500 µm. The consequence is an increase in pulmonary vascular resistance (PVR) and progressive right heart failure. [from ORDO]

Professional guidelines

PubMed

Evaluation and Management of Chronic Thromboembolic Pulmonary Hypertension.
Yang J, Madani MM, Mahmud E, Kim NH
Chest 2023 Aug;164(2):490-502. Epub 2023 Mar 28 doi: 10.1016/j.chest.2023.03.029. PMID: 36990148Free PMC Article
Pulmonary embolism: update on management and controversies.
Duffett L, Castellucci LA, Forgie MA
BMJ 2020 Aug 5;370:m2177. doi: 10.1136/bmj.m2177. PMID: 32759284
2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT).
Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, Simonneau G, Peacock A, Vonk Noordegraaf A, Beghetti M, Ghofrani A, Gomez Sanchez MA, Hansmann G, Klepetko W, Lancellotti P, Matucci M, McDonagh T, Pierard LA, Trindade PT, Zompatori M, Hoeper M; ESC Scientific Document Group
Eur Heart J 2016 Jan 1;37(1):67-119. Epub 2015 Aug 29 doi: 10.1093/eurheartj/ehv317. PMID: 26320113

Recent clinical studies

Etiology

Pulmonary hypertension - the latest updates for physicians.
Virsinskaite R, Karia N, Kotecha T, Schreiber BE, Coghlan JG, Knight DS
Clin Med (Lond) 2023 Sep;23(5):449-454. doi: 10.7861/clinmed.2023-23.5.Cardio4. PMID: 37775164Free PMC Article
Evaluation and Management of Chronic Thromboembolic Pulmonary Hypertension.
Yang J, Madani MM, Mahmud E, Kim NH
Chest 2023 Aug;164(2):490-502. Epub 2023 Mar 28 doi: 10.1016/j.chest.2023.03.029. PMID: 36990148Free PMC Article
Update on the management of venous thromboembolism.
Bartholomew JR
Cleve Clin J Med 2017 Dec;84(12 Suppl 3):39-46. doi: 10.3949/ccjm.84.s3.04. PMID: 29257737
Pulmonary Hypertension.
Hoeper MM, Ghofrani HA, Grünig E, Klose H, Olschewski H, Rosenkranz S
Dtsch Arztebl Int 2017 Feb 3;114(5):73-84. doi: 10.3238/arztebl.2017.0073. PMID: 28241922Free PMC Article
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

Pulmonary hypertension - the latest updates for physicians.
Virsinskaite R, Karia N, Kotecha T, Schreiber BE, Coghlan JG, Knight DS
Clin Med (Lond) 2023 Sep;23(5):449-454. doi: 10.7861/clinmed.2023-23.5.Cardio4. PMID: 37775164Free PMC Article
Evaluation and Management of Chronic Thromboembolic Pulmonary Hypertension.
Yang J, Madani MM, Mahmud E, Kim NH
Chest 2023 Aug;164(2):490-502. Epub 2023 Mar 28 doi: 10.1016/j.chest.2023.03.029. PMID: 36990148Free PMC Article
Chronic Thromboembolic Pulmonary Hypertension.
Teerapuncharoen K, Bag R
Lung 2022 Jun;200(3):283-299. Epub 2022 May 29 doi: 10.1007/s00408-022-00539-w. PMID: 35643802
Chronic Thromboembolic Pulmonary Hypertension.
Mullin CJ, Klinger JR
Heart Fail Clin 2018 Jul;14(3):339-351. doi: 10.1016/j.hfc.2018.02.009. PMID: 29966632
Pulmonary Hypertension.
Hoeper MM, Ghofrani HA, Grünig E, Klose H, Olschewski H, Rosenkranz S
Dtsch Arztebl Int 2017 Feb 3;114(5):73-84. doi: 10.3238/arztebl.2017.0073. PMID: 28241922Free PMC Article

Therapy

Evaluation and Management of Chronic Thromboembolic Pulmonary Hypertension.
Yang J, Madani MM, Mahmud E, Kim NH
Chest 2023 Aug;164(2):490-502. Epub 2023 Mar 28 doi: 10.1016/j.chest.2023.03.029. PMID: 36990148Free PMC Article
Regulation of VWF (Von Willebrand Factor) in Inflammatory Thrombosis.
Manz XD, Bogaard HJ, Aman J
Arterioscler Thromb Vasc Biol 2022 Nov;42(11):1307-1320. Epub 2022 Sep 29 doi: 10.1161/ATVBAHA.122.318179. PMID: 36172866
Chronic Thromboembolic Pulmonary Hypertension.
Teerapuncharoen K, Bag R
Lung 2022 Jun;200(3):283-299. Epub 2022 May 29 doi: 10.1007/s00408-022-00539-w. PMID: 35643802
Chronic thromboembolic pulmonary hypertension.
Kim NH, Delcroix M, Jais X, Madani MM, Matsubara H, Mayer E, Ogo T, Tapson VF, Ghofrani HA, Jenkins DP
Eur Respir J 2019 Jan;53(1) Epub 2019 Jan 24 doi: 10.1183/13993003.01915-2018. PMID: 30545969Free PMC Article
Update on the management of venous thromboembolism.
Bartholomew JR
Cleve Clin J Med 2017 Dec;84(12 Suppl 3):39-46. doi: 10.3949/ccjm.84.s3.04. PMID: 29257737

Prognosis

Pulmonary hypertension - the latest updates for physicians.
Virsinskaite R, Karia N, Kotecha T, Schreiber BE, Coghlan JG, Knight DS
Clin Med (Lond) 2023 Sep;23(5):449-454. doi: 10.7861/clinmed.2023-23.5.Cardio4. PMID: 37775164Free PMC Article
Pulmonary embolus.
Dix C, Tran H
Aust J Gen Pract 2022 Sep;51(9):667-671. doi: 10.31128/AJGP-05-22-6440. PMID: 36045622
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
Chronic Thromboembolic Pulmonary Hypertension.
Teerapuncharoen K, Bag R
Lung 2022 Jun;200(3):283-299. Epub 2022 May 29 doi: 10.1007/s00408-022-00539-w. PMID: 35643802
Diagnosis, Treatment and Follow Up of Acute Pulmonary Embolism: Consensus Practice from the PERT Consortium.
Rivera-Lebron B, McDaniel M, Ahrar K, Alrifai A, Dudzinski DM, Fanola C, Blais D, Janicke D, Melamed R, Mohrien K, Rozycki E, Ross CB, Klein AJ, Rali P, Teman NR, Yarboro L, Ichinose E, Sharma AM, Bartos JA, Elder M, Keeling B, Palevsky H, Naydenov S, Sen P, Amoroso N, Rodriguez-Lopez JM, Davis GA, Rosovsky R, Rosenfield K, Kabrhel C, Horowitz J, Giri JS, Tapson V, Channick R; PERT Consortium
Clin Appl Thromb Hemost 2019 Jan-Dec;25:1076029619853037. doi: 10.1177/1076029619853037. PMID: 31185730Free PMC Article

Clinical prediction guides

Single-Cell Analysis Reveals Distinct Immune and Smooth Muscle Cell Populations that Contribute to Chronic Thromboembolic Pulmonary Hypertension.
Viswanathan G, Kirshner HF, Nazo N, Ali S, Ganapathi A, Cumming I, Zhuang Y, Choi I, Warman A, Jassal C, Almeida-Peters S, Haney J, Corcoran D, Yu YR, Rajagopal S
Am J Respir Crit Care Med 2023 May 15;207(10):1358-1375. doi: 10.1164/rccm.202203-0441OC. PMID: 36803741Free PMC Article
Pulmonary embolus.
Dix C, Tran H
Aust J Gen Pract 2022 Sep;51(9):667-671. doi: 10.31128/AJGP-05-22-6440. PMID: 36045622
Chronic thromboembolic pulmonary hypertension.
Olsson KM, Meyer B, Hinrichs J, Vogel-Claussen J, Hoeper MM, Cebotari S
Dtsch Arztebl Int 2014 Dec 12;111(50):856-62. doi: 10.3238/arztebl.2014.0856. PMID: 25585582Free PMC Article
Chronic thromboembolic pulmonary hypertension.
Hoeper MM, Madani MM, Nakanishi N, Meyer B, Cebotari S, Rubin LJ
Lancet Respir Med 2014 Jul;2(7):573-82. Epub 2014 Jun 2 doi: 10.1016/S2213-2600(14)70089-X. PMID: 24898750
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

Anticoagulation in chronic thromboembolic pulmonary hypertension: A systematic review and meta-analysis.
Ishisaka Y, Watanabe A, Takagi H, Steiger D, Kuno T
Thromb Res 2023 Nov;231:91-98. Epub 2023 Oct 14 doi: 10.1016/j.thromres.2023.10.003. PMID: 37839150
Oxygen Therapy in Pulmonary Vascular Disease: A Systematic Review, Meta-Analysis, and Comment.
Schuster M, Müller J, Schwarz EI, Saxer S, Schneider SR, Ulrich S, Lichtblau M
Heart Fail Clin 2023 Jan;19(1S):e1-e11. Epub 2023 Feb 26 doi: 10.1016/j.hfc.2022.11.001. PMID: 36922056Free PMC Article
Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension: A Systematic Review and Meta-analysis.
Kennedy MK, Kennedy SA, Tan KT, de Perrot M, Bassett P, McInnis MC, Thenganatt J, Donahoe L, Granton J, Mafeld S
Cardiovasc Intervent Radiol 2023 Jan;46(1):5-18. Epub 2022 Dec 6 doi: 10.1007/s00270-022-03323-8. PMID: 36474104
Use of direct oral anticoagulants in chronic thromboembolic pulmonary hypertension: a systematic review.
Sedhom R, Megaly M, Gupta E, Amanullah A
J Thromb Thrombolysis 2022 Jan;53(1):51-57. Epub 2021 Jun 16 doi: 10.1007/s11239-021-02501-8. PMID: 34132973
Pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension : a systematic review.
Rahnavardi M, Yan TD, Cao C, Vallely MP, Bannon PG, Wilson MK
Ann Thorac Cardiovasc Surg 2011;17(5):435-45. Epub 2011 Jul 13 doi: 10.5761/atcs.oa.10.01653. PMID: 21881372

Supplemental Content

Table of contents

    Clinical resources

    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.

    Reviews

    Related information

    • OMIM
      Related records in OMIM
    • 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...