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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
Cardiorespiratory Interactions in Paediatrics: 'It's (almost always) the circulation stupid!'.
Rigby ML, Rosenthal M
Paediatr Respir Rev 2017 Mar;22:60-65. Epub 2016 Aug 12 doi: 10.1016/j.prrv.2016.08.003. PMID: 28029591
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

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
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
Fetal cardiology.
Tulzer G
Curr Opin Pediatr 2000 Oct;12(5):492-6. doi: 10.1097/00008480-200010000-00014. PMID: 11021416

Therapy

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
Pulmonary vascular involvement in COPD.
Peinado VI, Pizarro S, Barberà JA
Chest 2008 Oct;134(4):808-814. doi: 10.1378/chest.08-0820. PMID: 18842913

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
Stenting of the ductus arteriosus for ductal-dependent pulmonary blood flow-current techniques and procedural considerations.
Aggarwal V, Petit CJ, Glatz AC, Goldstein BH, Qureshi AM
Congenit Heart Dis 2019 Jan;14(1):110-115. doi: 10.1111/chd.12709. PMID: 30811792
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
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
Coronary-pulmonary artery fistula in tetralogy of Fallot with pulmonary atresia.
Talwar S, Anderson RH, Keshri VK, Choudhary SK, Gulati GS, Airan B
Asian Cardiovasc Thorac Ann 2014 Oct;22(8):1003-9. Epub 2013 Nov 12 doi: 10.1177/0218492313505101. PMID: 24887892

Clinical prediction guides

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
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
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
Biventricular repair versus uni-ventricular repair for pulmonary atresia with intact ventrical septum: A systematic review.
Li FF, Du XL, Chen S
J Huazhong Univ Sci Technolog Med Sci 2015 Oct;35(5):656-661. Epub 2015 Oct 22 doi: 10.1007/s11596-015-1485-3. PMID: 26489617
Bidirectional Glenn With Additional Pulmonary Blood Flow: Systematic Review and Evidence-Based Recommendations.
Alghamdi AA
J Card Surg 2015 Sep;30(9):724-30. Epub 2015 Jul 3 doi: 10.1111/jocs.12592. PMID: 26138594

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