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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
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

Surgical Strategies in Single Ventricle Management of Neonates and Infants.
Haller C, Barron DJ
Can J Cardiol 2022 Jul;38(7):909-920. Epub 2022 May 2 doi: 10.1016/j.cjca.2022.04.021. PMID: 35513174
Management of Fontan circulation in pregnancy: a multidisciplinary approach to care.
Wolfe NK, Sabol BA, Kelly JC, Dombrowski M, Benhardt AC, Fleckenstein J, Stout MJ, Lindley KJ
Am J Obstet Gynecol MFM 2021 Jan;3(1):100257. Epub 2020 Oct 17 doi: 10.1016/j.ajogmf.2020.100257. PMID: 33451613
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
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

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
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
The Fontan circulation after 45 years: update in physiology.
Gewillig M, Brown SC
Heart 2016 Jul 15;102(14):1081-6. Epub 2016 May 24 doi: 10.1136/heartjnl-2015-307467. PMID: 27220691Free PMC Article
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
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
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

Clinical prediction guides

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
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
The pathophysiology of chronic thromboembolic pulmonary hypertension.
Simonneau G, Torbicki A, Dorfmüller P, Kim N
Eur Respir Rev 2017 Mar 31;26(143) Epub 2017 Mar 29 doi: 10.1183/16000617.0112-2016. PMID: 28356405Free PMC Article
The Fontan circulation after 45 years: update in physiology.
Gewillig M, Brown SC
Heart 2016 Jul 15;102(14):1081-6. Epub 2016 May 24 doi: 10.1136/heartjnl-2015-307467. PMID: 27220691Free 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

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

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