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Abnormality of the pulmonary artery

MedGen UID:
488776
•Concept ID:
C0009681
•
Congenital Abnormality
Synonyms: Abnormality of lung artery; Anomalous pulmonary artery; Congenital anomaly of pulmonary artery; PA - Pulmonary artery abnormality; Pulmonary artery abnormality; Pulmonary artery congenital abnormality
SNOMED CT: Anomalous pulmonary artery (128588008); Pulmonary artery congenital abnormality (36110001); PA - Pulmonary artery abnormality (36110001); Pulmonary artery abnormality (36110001); Congenital anomaly of pulmonary artery (36110001)
 
HPO: HP:0004414

Definition

An abnormality of the pulmonary artery. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAbnormality of the pulmonary artery

Professional guidelines

PubMed

Pulmonary arterial hypertension: pathogenesis and clinical management.
Thenappan T, Ormiston ML, Ryan JJ, Archer SL
BMJ 2018 Mar 14;360:j5492. doi: 10.1136/bmj.j5492. PMID: 29540357Free PMC Article
Epidemiology, pathogenesis, and management of takotsubo syndrome.
Y-Hassan S, Tornvall P
Clin Auton Res 2018 Feb;28(1):53-65. Epub 2017 Sep 15 doi: 10.1007/s10286-017-0465-z. PMID: 28917022Free PMC Article
Alagille syndrome: pathogenesis, diagnosis and management.
Turnpenny PD, Ellard S
Eur J Hum Genet 2012 Mar;20(3):251-7. Epub 2011 Sep 21 doi: 10.1038/ejhg.2011.181. PMID: 21934706Free PMC Article

Recent clinical studies

Etiology

Staged unifocalization revisited.
Nakamura Y, Burkhart HM
J Card Surg 2022 Apr;37(4):967-968. Epub 2022 Feb 9 doi: 10.1111/jocs.16296. PMID: 35137983
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
Congenital anomalies of the pulmonary arteries: an imaging overview.
Escalon JG, Browne LP, Bang TJ, Restrepo CS, Ocazionez D, Vargas D
Br J Radiol 2019 Jan;92(1093):20180185. Epub 2018 Aug 31 doi: 10.1259/bjr.20180185. PMID: 30102560Free PMC Article
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
Discontinuous Pulmonary Artery.
Eltayeb O, Mongé MC, Popescu AR, Sarwark AE, Harris T, Backer CL
World J Pediatr Congenit Heart Surg 2017 Jan;8(1):106-110. Epub 2016 Jun 22 doi: 10.1177/2150135115618871. PMID: 26993757

Diagnosis

Prenatal screening and diagnosis of pulmonary artery anomalies.
Lesieur E, Zaffran S, Chaoui R, Quarello E
Ultrasound Obstet Gynecol 2023 Apr;61(4):445-457. doi: 10.1002/uog.26078. PMID: 36178851
Pulmonary Arteriovenous Malformation and Its Vascular Mimickers.
Lee HN, Hyun D
Korean J Radiol 2022 Feb;23(2):202-217. Epub 2022 Jan 4 doi: 10.3348/kjr.2021.0417. PMID: 35029077Free PMC Article
Congenital anomalies of the pulmonary arteries: an imaging overview.
Escalon JG, Browne LP, Bang TJ, Restrepo CS, Ocazionez D, Vargas D
Br J Radiol 2019 Jan;92(1093):20180185. Epub 2018 Aug 31 doi: 10.1259/bjr.20180185. PMID: 30102560Free PMC Article
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
Discontinuous Pulmonary Artery.
Eltayeb O, Mongé MC, Popescu AR, Sarwark AE, Harris T, Backer CL
World J Pediatr Congenit Heart Surg 2017 Jan;8(1):106-110. Epub 2016 Jun 22 doi: 10.1177/2150135115618871. PMID: 26993757

Therapy

Diagnosis and Management of Persistent Pulmonary Arterio-venous Malformations following Embolotherapy.
Kulkarni CB, Sutphin P, Iqbal S, Kalva SP
Acad Radiol 2023 Mar;30(3):441-452. Epub 2022 Sep 9 doi: 10.1016/j.acra.2022.08.012. PMID: 36089478
Predictive Models for Pulmonary Artery Size in Fontan Patients.
Gupta A, Gillett C, Gerard P, Cheung MMH, Mynard JP, Kung E
J Cardiovasc Transl Res 2021 Aug;14(4):782-789. Epub 2020 Apr 4 doi: 10.1007/s12265-020-09993-4. PMID: 32248348
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
Embolisation for pulmonary arteriovenous malformation.
Hsu CC, Kwan GN, Evans-Barns H, van Driel ML
Cochrane Database Syst Rev 2018 Jan 4;1(1):CD008017. doi: 10.1002/14651858.CD008017.pub5. PMID: 29298459Free PMC Article
Pulmonary arteriovenous malformations after the superior cavopulmonary shunt: mechanisms and clinical implications.
Kavarana MN, Jones JA, Stroud RE, Bradley SM, Ikonomidis JS, Mukherjee R
Expert Rev Cardiovasc Ther 2014 Jun;12(6):703-13. Epub 2014 Apr 23 doi: 10.1586/14779072.2014.912132. PMID: 24758411Free PMC Article

Prognosis

Prenatal screening and diagnosis of pulmonary artery anomalies.
Lesieur E, Zaffran S, Chaoui R, Quarello E
Ultrasound Obstet Gynecol 2023 Apr;61(4):445-457. doi: 10.1002/uog.26078. PMID: 36178851
Congenital anomalies of the pulmonary arteries: an imaging overview.
Escalon JG, Browne LP, Bang TJ, Restrepo CS, Ocazionez D, Vargas D
Br J Radiol 2019 Jan;92(1093):20180185. Epub 2018 Aug 31 doi: 10.1259/bjr.20180185. PMID: 30102560Free PMC Article
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 arteriovenous malformations.
Cartin-Ceba R, Swanson KL, Krowka MJ
Chest 2013 Sep;144(3):1033-1044. doi: 10.1378/chest.12-0924. PMID: 24008954
Coronary artery fistulae.
Luo L, Kebede S, Wu S, Stouffer GA
Am J Med Sci 2006 Aug;332(2):79-84. doi: 10.1097/00000441-200608000-00005. PMID: 16909054

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
Congenital abnormalities of the aortic arch: revisiting the 1964 Stewart classification.
Li S, Wen H, Liang M, Luo D, Qin Y, Liao Y, Ouyang S, Bi J, Tian X, Norwitz ER, Luo G
Cardiovasc Pathol 2019 Mar-Apr;39:38-50. Epub 2018 Dec 6 doi: 10.1016/j.carpath.2018.11.004. PMID: 30623879
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

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
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
Internal mammary artery-to-pulmonary vasculature fistula: Systematic review of case reports.
Abdul Jabbar A, Patel A, Marzlin N, Altabaqchali S, Hasan M, Al-Zubaidi M, Agarwal A
Vasc Med 2017 Oct;22(5):426-431. doi: 10.1177/1358863X17724262. PMID: 28990495
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
Coronary-Pulmonary Artery Fistulas: A Systematic Review.
Verdini D, Vargas D, Kuo A, Ghoshhajra B, Kim P, Murillo H, Kirsch J, Lane M, Restrepo C
J Thorac Imaging 2016 Nov;31(6):380-390. doi: 10.1097/RTI.0000000000000232. PMID: 27768631

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