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Congenital stenosis of pulmonary valve

MedGen UID:
511533
Concept ID:
C0162164
Congenital Abnormality
Synonym: Congenital pulmonic valve stenosis
SNOMED CT: Congenital stenosis of pulmonary valve (67278007); Congenital valvular pulmonic stenosis (67278007); PV - Congenital pulmonary valve stenosis (67278007)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
Concept ID:
C0600599
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0017865
Orphanet: ORPHA3189

Definition

A rare congenital heart malformation characterized by an obstruction to flow through the pulmonary valve with a clinical presentation that may vary from critical stenosis presenting in the neonatal period to asymptomatic mild stenosis. The obstruction at the valvular level can be associated with obstruction at the subpulmonary, or supravalvar levels (valvar, subpulmonary, supravalvar pulmonary stenosis (PS). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital stenosis of pulmonary valve
Follow this link to review classifications for Congenital stenosis of pulmonary valve in Orphanet.

Professional guidelines

PubMed

Marchini F, Meossi S, Passarini G, Campo G, Pavasini R
Vasc Health Risk Manag 2023;19:379-390. Epub 2023 Jun 30 doi: 10.2147/VHRM.S380240. PMID: 37416511Free PMC Article
Expert Consensus Panel:, Miller JR, Stephens EH, Goldstone AB, Glatz AC, Kane L, Van Arsdell GS, Stellin G, Barron DJ, d'Udekem Y, Benson L, Quintessenza J, Ohye RG, Talwar S, Fremes SE, Emani SM, Eghtesady P
J Thorac Cardiovasc Surg 2023 Jan;165(1):221-250. Epub 2022 Oct 26 doi: 10.1016/j.jtcvs.2022.07.025. PMID: 36522807
Linglart L, Gelb BD
Am J Med Genet C Semin Med Genet 2020 Mar;184(1):73-80. Epub 2020 Feb 5 doi: 10.1002/ajmg.c.31765. PMID: 32022400Free PMC Article

Recent clinical studies

Etiology

Expert Consensus Panel:, Miller JR, Stephens EH, Goldstone AB, Glatz AC, Kane L, Van Arsdell GS, Stellin G, Barron DJ, d'Udekem Y, Benson L, Quintessenza J, Ohye RG, Talwar S, Fremes SE, Emani SM, Eghtesady P
J Thorac Cardiovasc Surg 2023 Jan;165(1):221-250. Epub 2022 Oct 26 doi: 10.1016/j.jtcvs.2022.07.025. PMID: 36522807
Bouhlel I, Ajmi H, Slim M, Gribaa R, Naffati E
Tunis Med 2021 Feb;99(2):291-297. PMID: 33899201Free PMC Article
Morray BH, McElhinney DB
J Am Coll Cardiol 2021 Jan 5;77(1):71-79. doi: 10.1016/j.jacc.2020.10.052. PMID: 33413944
Balzer D
Methodist Debakey Cardiovasc J 2019 Apr-Jun;15(2):122-132. doi: 10.14797/mdcj-15-2-122. PMID: 31384375Free PMC Article
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

Diagnosis

Kovacs S, Scansen BA, Stern JA
Vet Clin North Am Small Anim Pract 2023 Nov;53(6):1379-1391. Epub 2023 Jul 7 doi: 10.1016/j.cvsm.2023.05.014. PMID: 37423844
Marchini F, Meossi S, Passarini G, Campo G, Pavasini R
Vasc Health Risk Manag 2023;19:379-390. Epub 2023 Jun 30 doi: 10.2147/VHRM.S380240. PMID: 37416511Free PMC Article
Bouhlel I, Ajmi H, Slim M, Gribaa R, Naffati E
Tunis Med 2021 Feb;99(2):291-297. PMID: 33899201Free PMC Article
Fishbein GA, Fishbein MC
Curr Cardiol Rep 2019 May 18;21(7):54. doi: 10.1007/s11886-019-1143-7. PMID: 31104143
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

Therapy

Expert Consensus Panel:, Miller JR, Stephens EH, Goldstone AB, Glatz AC, Kane L, Van Arsdell GS, Stellin G, Barron DJ, d'Udekem Y, Benson L, Quintessenza J, Ohye RG, Talwar S, Fremes SE, Emani SM, Eghtesady P
J Thorac Cardiovasc Surg 2023 Jan;165(1):221-250. Epub 2022 Oct 26 doi: 10.1016/j.jtcvs.2022.07.025. PMID: 36522807
Friedman KG, Tworetzky W
Arch Cardiovasc Dis 2020 Feb;113(2):121-128. Epub 2020 Feb 26 doi: 10.1016/j.acvd.2019.06.007. PMID: 32113817
Balzer D
Methodist Debakey Cardiovasc J 2019 Apr-Jun;15(2):122-132. doi: 10.14797/mdcj-15-2-122. PMID: 31384375Free PMC Article
Kheiwa A, Divanji P, Mahadevan VS
Expert Rev Cardiovasc Ther 2018 Mar;16(3):197-207. Epub 2018 Feb 12 doi: 10.1080/14779072.2018.1435273. PMID: 29433351
Hijazi ZM, Awad SM
JACC Cardiovasc Interv 2008 Dec;1(6):603-11. doi: 10.1016/j.jcin.2008.07.007. PMID: 19463373

Prognosis

Bouhlel I, Ajmi H, Slim M, Gribaa R, Naffati E
Tunis Med 2021 Feb;99(2):291-297. PMID: 33899201Free PMC Article
Faries CM, Sengupta A, Alexis SL, Tadros RO, Octeau D, Barnes HJ, Tang GHL
Surg Technol Int 2020 May 28;36:217-223. PMID: 32379893
van der Ven JPG, van den Bosch E, Bogers AJCC, Helbing WA
F1000Res 2019;8 Epub 2019 Aug 29 doi: 10.12688/f1000research.17174.1. PMID: 31508203Free PMC Article
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
Latson LA
J Interv Cardiol 2001 Jun;14(3):345-50. doi: 10.1111/j.1540-8183.2001.tb00343.x. PMID: 12053395

Clinical prediction guides

Expert Consensus Panel:, Miller JR, Stephens EH, Goldstone AB, Glatz AC, Kane L, Van Arsdell GS, Stellin G, Barron DJ, d'Udekem Y, Benson L, Quintessenza J, Ohye RG, Talwar S, Fremes SE, Emani SM, Eghtesady P
J Thorac Cardiovasc Surg 2023 Jan;165(1):221-250. Epub 2022 Oct 26 doi: 10.1016/j.jtcvs.2022.07.025. PMID: 36522807
Bouhlel I, Ajmi H, Slim M, Gribaa R, Naffati E
Tunis Med 2021 Feb;99(2):291-297. PMID: 33899201Free PMC Article
Minnella GP, Crupano FM, Syngelaki A, Zidere V, Akolekar R, Nicolaides KH
Ultrasound Obstet Gynecol 2020 May;55(5):637-644. doi: 10.1002/uog.21956. PMID: 31875326
van der Ven JPG, van den Bosch E, Bogers AJCC, Helbing WA
F1000Res 2019;8 Epub 2019 Aug 29 doi: 10.12688/f1000research.17174.1. PMID: 31508203Free PMC Article
Pessel C, Bonanno C
Semin Perinatol 2014 Aug;38(5):273-84. Epub 2014 Jun 25 doi: 10.1053/j.semperi.2014.04.016. PMID: 25037517

Recent systematic reviews

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
Diniz AMB, Manso PH, Santos MV, Rodrigues AJ, Sbragia L
Braz J Cardiovasc Surg 2023 May 4;38(3):398-404. doi: 10.21470/1678-9741-2022-0273. PMID: 36592074Free PMC Article
Shivaram P, Van den Eynde J, Barnes BT, Danford DA, Cedars A, Kutty S
Fetal Diagn Ther 2022;49(5-6):225-234. Epub 2022 Jul 6 doi: 10.1159/000525718. PMID: 35793649
Ravindra K, Chanana N, Mor S
Sci Total Environ 2021 Apr 15;765:142772. Epub 2020 Oct 7 doi: 10.1016/j.scitotenv.2020.142772. PMID: 33183823
Cai GJ, Sun XX, Zhang L, Hong Q
Am J Obstet Gynecol 2014 Aug;211(2):91-117. Epub 2014 Mar 14 doi: 10.1016/j.ajog.2014.03.028. PMID: 24631708

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