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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
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
Pediatric Pulmonary Hypertension: Guidelines From the American Heart Association and American Thoracic Society.
Abman SH, Hansmann G, Archer SL, Ivy DD, Adatia I, Chung WK, Hanna BD, Rosenzweig EB, Raj JU, Cornfield D, Stenmark KR, Steinhorn R, Thébaud B, Fineman JR, Kuehne T, Feinstein JA, Friedberg MK, Earing M, Barst RJ, Keller RL, Kinsella JP, Mullen M, Deterding R, Kulik T, Mallory G, Humpl T, Wessel DL; American Heart Association Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation; Council on Clinical Cardiology; Council on Cardiovascular Disease in the Young; Council on Cardiovascular Radiology and Intervention; Council on Cardiovascular Surgery and Anesthesia; and the American Thoracic Society
Circulation 2015 Nov 24;132(21):2037-99. Epub 2015 Nov 3 doi: 10.1161/CIR.0000000000000329. PMID: 26534956

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
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

Diagnosis

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
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
The Lung in Hereditary Hemorrhagic Telangiectasia.
Dupuis-Girod S, Cottin V, Shovlin CL
Respiration 2017;94(4):315-330. Epub 2017 Aug 30 doi: 10.1159/000479632. PMID: 28850955
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
Scimitar syndrome.
Ielasi A, Latib A, Agricola E, Montorfano M, Colombo A
J Cardiovasc Med (Hagerstown) 2011 Mar;12(3):176-7. doi: 10.2459/JCM.0b013e328332f3e2. PMID: 20093947

Therapy

What "FUEL"s the Fontan circulation-solvitur ambulando!
Rychik J
J Thorac Cardiovasc Surg 2021 Oct;162(4):1234-1238. Epub 2020 Sep 15 doi: 10.1016/j.jtcvs.2020.08.114. PMID: 33618865
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

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
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
Scimitar syndrome.
Ielasi A, Latib A, Agricola E, Montorfano M, Colombo A
J Cardiovasc Med (Hagerstown) 2011 Mar;12(3):176-7. doi: 10.2459/JCM.0b013e328332f3e2. PMID: 20093947

Recent systematic reviews

Pulmonary vasodilators and exercise in Fontan circulation: a systematic review and meta-analysis.
Kosmidis D, Arvanitaki A, Farmakis IT, Liakos A, Giannopoulos A, Ziakas A, Giannakoulas G
Heart 2024 Mar 22;110(8):552-559. doi: 10.1136/heartjnl-2023-323166. PMID: 37918902
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
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

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