U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Abnormality of pulmonary circulation

MedGen UID:
908799
•Concept ID:
C4280730
•
Finding
Synonyms: Abnormal pulmonary circulation; Abnormality of respiratory circulation
 
HPO: HP:0030875

Definition

A functional anomaly of that portion of the cardiosvascular system that carries deoxygenated blood from the heart to the lungs and returns oxygenated blood back to the heart. [from HPO]

Professional guidelines

PubMed

Pathophysiology and Management of Persistent Pulmonary Hypertension of the Newborn.
Singh Y, Lakshminrusimha S
Clin Perinatol 2021 Aug;48(3):595-618. doi: 10.1016/j.clp.2021.05.009. PMID: 34353582Free PMC Article
Hyponatremia in Heart Failure: Pathogenesis and Management.
Rodriguez M, Hernandez M, Cheungpasitporn W, Kashani KB, Riaz I, Rangaswami J, Herzog E, Guglin M, Krittanawong C
Curr Cardiol Rev 2019;15(4):252-261. doi: 10.2174/1573403X15666190306111812. PMID: 30843491Free PMC Article
Evaluation and Management of Right-Sided Heart Failure: A Scientific Statement From the American Heart Association.
Konstam MA, Kiernan MS, Bernstein D, Bozkurt B, Jacob M, Kapur NK, Kociol RD, Lewis EF, Mehra MR, Pagani FD, Raval AN, Ward C; American Heart Association Council on Clinical Cardiology; Council on Cardiovascular Disease in the Young; and Council on Cardiovascular Surgery and Anesthesia
Circulation 2018 May 15;137(20):e578-e622. Epub 2018 Apr 12 doi: 10.1161/CIR.0000000000000560. PMID: 29650544

Recent clinical studies

Etiology

Pulmonary arteriovenous malformations.
Tellapuri S, Park HS, Kalva SP
Int J Cardiovasc Imaging 2019 Aug;35(8):1421-1428. Epub 2018 Nov 1 doi: 10.1007/s10554-018-1479-x. PMID: 30386957
State-of-the-art pulmonary arterial imaging - Part 2.
Goerne H, Chaturvedi A, Partovi S, Rajiah P
Vasa 2018 Aug;47(5):361-375. Epub 2018 May 28 doi: 10.1024/0301-1526/a000709. PMID: 29806799
Pulmonary vascular development in congenital diaphragmatic hernia.
Mous DS, Kool HM, Wijnen R, Tibboel D, Rottier RJ
Eur Respir Rev 2018 Mar 31;27(147) Epub 2018 Jan 24 doi: 10.1183/16000617.0104-2017. PMID: 29367409Free PMC Article
Patent foramen ovale.
Homma S, Messé SR, Rundek T, Sun YP, Franke J, Davidson K, Sievert H, Sacco RL, Di Tullio MR
Nat Rev Dis Primers 2016 Jan 21;2:15086. doi: 10.1038/nrdp.2015.86. PMID: 27188965
Fetal cardiology.
Tulzer G
Curr Opin Pediatr 2000 Oct;12(5):492-6. doi: 10.1097/00008480-200010000-00014. PMID: 11021416

Diagnosis

Late deaths after Fontan procedure: the next frontier.
Luo S, Honjo O
Curr Opin Cardiol 2019 Mar;34(2):156-163. doi: 10.1097/HCO.0000000000000603. PMID: 30575650
Pulmonary arteriovenous malformations.
Tellapuri S, Park HS, Kalva SP
Int J Cardiovasc Imaging 2019 Aug;35(8):1421-1428. Epub 2018 Nov 1 doi: 10.1007/s10554-018-1479-x. PMID: 30386957
State-of-the-art pulmonary arterial imaging - Part 2.
Goerne H, Chaturvedi A, Partovi S, Rajiah P
Vasa 2018 Aug;47(5):361-375. Epub 2018 May 28 doi: 10.1024/0301-1526/a000709. PMID: 29806799
Understanding cardiac shunts.
Joffe DC, Shi MR, Welker CC
Paediatr Anaesth 2018 Apr;28(4):316-325. Epub 2018 Mar 6 doi: 10.1111/pan.13347. PMID: 29508477
Hypoxic Pulmonary Vasoconstriction: From Molecular Mechanisms to Medicine.
Dunham-Snary KJ, Wu D, Sykes EA, Thakrar A, Parlow LRG, Mewburn JD, Parlow JL, Archer SL
Chest 2017 Jan;151(1):181-192. Epub 2016 Sep 16 doi: 10.1016/j.chest.2016.09.001. PMID: 27645688Free PMC Article

Therapy

The Fontan Circulation: From Ideal to Failing Hemodynamics and Drug Therapies for Optimization.
Perrin N, Dore A, van de Bruaene A, Mongeon FP, Mondésert B, Poirier N, Miró J, Khairy P, Ibrahim R, Chaix MA
Can J Cardiol 2022 Jul;38(7):1059-1071. Epub 2022 Apr 23 doi: 10.1016/j.cjca.2022.04.014. PMID: 35469974
Pulmonary arteriovenous malformations.
Tellapuri S, Park HS, Kalva SP
Int J Cardiovasc Imaging 2019 Aug;35(8):1421-1428. Epub 2018 Nov 1 doi: 10.1007/s10554-018-1479-x. PMID: 30386957
Effect of antidepressants use in pregnancy on foetus development and adverse effects in newborns.
Bałkowiec-Iskra E, Mirowska-Guzel DM, Wielgoś M
Ginekol Pol 2017;88(1):36-42. doi: 10.5603/GP.a2017.0007. PMID: 28157249
Pulmonary vasodilator therapy in the failing Fontan circulation: rationale and efficacy.
Snarr BS, Paridon SM, Rychik J, Goldberg DJ
Cardiol Young 2015 Dec;25(8):1489-92. doi: 10.1017/S1047951115002309. PMID: 26675595
Fontan physiology revisited.
Jolley M, Colan SD, Rhodes J, DiNardo J
Anesth Analg 2015 Jul;121(1):172-182. doi: 10.1213/ANE.0000000000000717. PMID: 26086514

Prognosis

Cross-sectional imaging of congenital pulmonary artery anomalies.
Zucker EJ
Int J Cardiovasc Imaging 2019 Aug;35(8):1535-1548. Epub 2019 Jun 7 doi: 10.1007/s10554-019-01643-4. PMID: 31175525
State-of-the-art pulmonary arterial imaging - Part 2.
Goerne H, Chaturvedi A, Partovi S, Rajiah P
Vasa 2018 Aug;47(5):361-375. Epub 2018 May 28 doi: 10.1024/0301-1526/a000709. PMID: 29806799
Pulmonary vascular development in congenital diaphragmatic hernia.
Mous DS, Kool HM, Wijnen R, Tibboel D, Rottier RJ
Eur Respir Rev 2018 Mar 31;27(147) Epub 2018 Jan 24 doi: 10.1183/16000617.0104-2017. PMID: 29367409Free PMC Article
Congenital Malformations Leading to Paradoxical Embolism.
Bruckheimer E
Cardiol Clin 2016 May;34(2):247-54. Epub 2016 Mar 10 doi: 10.1016/j.ccl.2015.12.005. PMID: 27150173
Right heart and pulmonary vessels structure and function.
D'Alto M, Scognamiglio G, Dimopoulos K, Bossone E, Vizza D, Romeo E, Vonk-Noordergraaf A, Gaine S, Peacock A, Naeije R
Echocardiography 2015 Jan;32 Suppl 1:S3-10. Epub 2014 Sep 19 doi: 10.1111/echo.12227. PMID: 25244348

Clinical prediction guides

Exercise Testing in the Risk Assessment of Pulmonary Hypertension.
Forbes LM, Bull TM, Lahm T, Make BJ, Cornwell WK 3rd
Chest 2023 Sep;164(3):736-746. Epub 2023 Apr 14 doi: 10.1016/j.chest.2023.04.013. PMID: 37061028Free PMC Article
Cross-sectional imaging of congenital pulmonary artery anomalies.
Zucker EJ
Int J Cardiovasc Imaging 2019 Aug;35(8):1535-1548. Epub 2019 Jun 7 doi: 10.1007/s10554-019-01643-4. PMID: 31175525
Update on MR imaging of the pulmonary vasculature.
Aziz M, Krishnam M, Madhuranthakam AJ, Rajiah P
Int J Cardiovasc Imaging 2019 Aug;35(8):1483-1497. Epub 2019 Apr 27 doi: 10.1007/s10554-019-01603-y. PMID: 31030315
State-of-the-art pulmonary arterial imaging - Part 2.
Goerne H, Chaturvedi A, Partovi S, Rajiah P
Vasa 2018 Aug;47(5):361-375. Epub 2018 May 28 doi: 10.1024/0301-1526/a000709. PMID: 29806799
State-of-the-art pulmonary arterial imaging - Part 1.
Tanabe Y, Landeras L, Ghandour A, Partovi S, Rajiah P
Vasa 2018 Aug;47(5):345-359. Epub 2018 May 28 doi: 10.1024/0301-1526/a000708. PMID: 29806797

Recent systematic reviews

Prediction of postnatal circulation in pulmonary atresia/critical stenosis with intact ventricular septum: systematic review and external validation of models.
Villalaín C, Moon-Grady AJ, Herberg U, Strainic J, Cohen JL, Shah A, Levi DS, Gómez-Montes E, Herraiz I, Galindo A
Ultrasound Obstet Gynecol 2023 Jul;62(1):14-22. Epub 2023 Jun 5 doi: 10.1002/uog.26176. PMID: 36776132
Patent Ductus Arteriosus Stent Versus Surgical Aortopulmonary Shunt for Initial Palliation of Cyanotic Congenital Heart Disease with Ductal-Dependent Pulmonary Blood Flow: A Systematic Review and Meta-Analysis.
Tseng SY, Truong VT, Peck D, Kandi S, Brayer S, Jason DP 3rd, Mazur W, Hill GD, Ashfaq A, Goldstein BH, Alsaied T
J Am Heart Assoc 2022 Jul 5;11(13):e024721. Epub 2022 Jun 29 doi: 10.1161/JAHA.121.024721. PMID: 35766251Free PMC Article
Blalock-Taussig Shunt versus Ductal Stent in the Palliation of Duct Dependent Pulmonary Circulation; A Systematic Review and Metanalysis.
Fakhry AbdelMassih A, Menshawey R, Menshawey E, El-Maghraby AE, Sabry AO, Kamel A, Seyam MY, Elshawarbi P, Ahmed SN, Salem SS, Ragab S, Nada Y, Agha H
Curr Probl Cardiol 2022 Sep;47(9):100885. Epub 2021 May 8 doi: 10.1016/j.cpcardiol.2021.100885. PMID: 34175152
Fate of Patients With Single Ventricles Who Do Not Undergo the Fontan Procedure.
Zheng WC, Lee MGY, d'Udekem Y
Ann Thorac Surg 2022 Jul;114(1):25-33. Epub 2021 Feb 17 doi: 10.1016/j.athoracsur.2021.02.011. PMID: 33609544
Risk Factors for Coarctation of the Aorta on Prenatal Ultrasound: A Systematic Review and Meta-Analysis.
Familiari A, Morlando M, Khalil A, Sonesson SE, Scala C, Rizzo G, Del Sordo G, Vassallo C, Elena Flacco M, Manzoli L, Lanzone A, Scambia G, Acharya G, D'Antonio F
Circulation 2017 Feb 21;135(8):772-785. Epub 2016 Dec 29 doi: 10.1161/CIRCULATIONAHA.116.024068. PMID: 28034902

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.

    Consumer resources

    Reviews

    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...