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Pulmonary valve stenosis

MedGen UID:
18768
•Concept ID:
C0034089
•
Disease or Syndrome
Synonyms: Pulmonary Valve Stenoses; Pulmonary Valve Stenosis; Pulmonic Stenoses, Valvular; Pulmonic Stenosis, Valvular; Stenoses, Pulmonary Valve; Stenosis, Pulmonary Valve; Valvular Pulmonic Stenoses; Valvular Pulmonic Stenosis
SNOMED CT: PVS - Pulmonary valve stenosis (56786000); PS - Pulmonary valve stenosis (56786000); Pulmonic valve stenosis (56786000); Pulmonary valve stenosis (56786000)
 
Monarch Initiative: MONDO:0006936

Definition

The pathologic narrowing of the orifice of the pulmonary valve. This lesion restricts blood outflow from the right ventricle to the pulmonary artery. When the trileaflet valve is fused into an imperforate membrane, the blockage is complete. [from MONDO]

Professional guidelines

PubMed

Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniques and Future Prospects.
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
Congenital heart defects in Noonan syndrome: Diagnosis, management, and treatment.
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
Pulmonary valve stenosis in the adult patient: pathophysiology, diagnosis and management.
Ruckdeschel E, Kim YY
Heart 2019 Mar;105(5):414-422. Epub 2018 Oct 18 doi: 10.1136/heartjnl-2017-312743. PMID: 30337335

Curated

UK NICE Guideline NG208, Heart valve disease presenting in adults: investigation and management, 2021

Recent clinical studies

Etiology

Preserving the pulmonary valve in Tetralogy of Fallot repair: Reconsidering the indication for valve-sparing.
Siddiqi U, Adewale A, Pena E, Schulz K, Ilbawi M, El-Zein C, Vricella L, Hibino N
J Card Surg 2022 Dec;37(12):5144-5152. Epub 2022 Nov 15 doi: 10.1111/jocs.17156. PMID: 36378940Free PMC Article
Outcome and right ventricle remodelling after valve replacement for pulmonic stenosis.
Laflamme E, Wald RM, Roche SL, Silversides CK, Thorne SA, Colman JM, Benson L, Osten M, Horlick E, Oechslin E, Alonso-Gonzalez R
Heart 2022 Jul 27;108(16):1290-1295. doi: 10.1136/heartjnl-2021-320121. PMID: 34815333
Pulmonary Valve Replacement for Tetralogy of Fallot.
Balzer D
Methodist Debakey Cardiovasc J 2019 Apr-Jun;15(2):122-132. doi: 10.14797/mdcj-15-2-122. PMID: 31384375Free PMC Article
Pulmonary valve interventions.
Odenwald T, Taylor AM
Expert Rev Cardiovasc Ther 2011 Nov;9(11):1445-57. doi: 10.1586/erc.11.150. PMID: 22059793
Critical pulmonary stenosis.
Latson LA
J Interv Cardiol 2001 Jun;14(3):345-50. doi: 10.1111/j.1540-8183.2001.tb00343.x. PMID: 12053395

Diagnosis

The Genetics of Canine Pulmonary Valve Stenosis.
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
Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniques and Future Prospects.
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
Pulmonary valve stenosis in the adult patient: pathophysiology, diagnosis and management.
Ruckdeschel E, Kim YY
Heart 2019 Mar;105(5):414-422. Epub 2018 Oct 18 doi: 10.1136/heartjnl-2017-312743. PMID: 30337335
Critical pulmonary stenosis.
Latson LA
J Interv Cardiol 2001 Jun;14(3):345-50. doi: 10.1111/j.1540-8183.2001.tb00343.x. PMID: 12053395
Pulmonary valvular stenosis.
Lucas RV Jr, Moller JH
Cardiovasc Clin 1970;2(1):155-84. PMID: 4938797

Therapy

Outcome and right ventricle remodelling after valve replacement for pulmonic stenosis.
Laflamme E, Wald RM, Roche SL, Silversides CK, Thorne SA, Colman JM, Benson L, Osten M, Horlick E, Oechslin E, Alonso-Gonzalez R
Heart 2022 Jul 27;108(16):1290-1295. doi: 10.1136/heartjnl-2021-320121. PMID: 34815333
Semilunar Valve Interventions for Congenital Heart Disease: JACC State-of-the-Art Review.
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
Pulmonary Valve Replacement for Tetralogy of Fallot.
Balzer D
Methodist Debakey Cardiovasc J 2019 Apr-Jun;15(2):122-132. doi: 10.14797/mdcj-15-2-122. PMID: 31384375Free PMC Article
Transcatheter pulmonary valvulation: current indications and available devices.
Hascoët S, Acar P, Boudjemline Y
Arch Cardiovasc Dis 2014 Nov;107(11):625-34. Epub 2014 Oct 31 doi: 10.1016/j.acvd.2014.07.048. PMID: 25444020
Critical pulmonary stenosis.
Latson LA
J Interv Cardiol 2001 Jun;14(3):345-50. doi: 10.1111/j.1540-8183.2001.tb00343.x. PMID: 12053395

Prognosis

30 years' experience with the arterial switch operation: risk of pulmonary stenosis and its impact on post-operative prognosis.
Sobczak-Budlewska K, Łubisz M, Moll M, Moszura T, Moll JA, Korabiewska-Pluta S, Moll JJ, Michalak KW
Cardiol Young 2023 Sep;33(9):1550-1555. Epub 2022 Aug 30 doi: 10.1017/S1047951122002670. PMID: 36040409
Determinants of long-term outcome of repaired pulmonary valve stenosis.
Galian-Gay L, Gordon B, Marsal JR, Rafecas A, Pijuan Domènech A, Castro MA, Subirana Domènech MT, Sureda C, Miranda B, Martí G, García-Dorado D, Dos Subirà L
Rev Esp Cardiol (Engl Ed) 2020 Feb;73(2):131-138. Epub 2019 May 24 doi: 10.1016/j.rec.2019.02.014. PMID: 31130344
Critical pulmonary stenosis.
Latson LA
J Interv Cardiol 2001 Jun;14(3):345-50. doi: 10.1111/j.1540-8183.2001.tb00343.x. PMID: 12053395
Balloon valvuloplasty for pulmonary valve stenosis.
Fedderly RT, Beekman RH 3rd
J Interv Cardiol 1995 Oct;8(5):451-61. doi: 10.1111/j.1540-8183.1995.tb00575.x. PMID: 10159512
Percutaneous balloon valvuloplasty.
Nishimura RA, Holmes DR Jr, Reeder GS
Mayo Clin Proc 1990 Feb;65(2):198-220. doi: 10.1016/s0025-6196(12)65015-0. PMID: 2406521

Clinical prediction guides

Molecular and phenotypic spectrum of cardio-facio-cutaneous syndrome in Chinese patients.
Feng B, Li X, Zhang Q, Wang Y, Gu S, Yao RE, Li Z, Gao S, Chang G, Li Q, Li N, Fu L, Wang J, Wang X
Orphanet J Rare Dis 2023 Sep 11;18(1):284. doi: 10.1186/s13023-023-02878-0. PMID: 37697378Free PMC Article
Preserving the pulmonary valve in Tetralogy of Fallot repair: Reconsidering the indication for valve-sparing.
Siddiqi U, Adewale A, Pena E, Schulz K, Ilbawi M, El-Zein C, Vricella L, Hibino N
J Card Surg 2022 Dec;37(12):5144-5152. Epub 2022 Nov 15 doi: 10.1111/jocs.17156. PMID: 36378940Free PMC Article
Outcome and right ventricle remodelling after valve replacement for pulmonic stenosis.
Laflamme E, Wald RM, Roche SL, Silversides CK, Thorne SA, Colman JM, Benson L, Osten M, Horlick E, Oechslin E, Alonso-Gonzalez R
Heart 2022 Jul 27;108(16):1290-1295. doi: 10.1136/heartjnl-2021-320121. PMID: 34815333
Supravalvular and Valvular Pulmonary Stenosis: Predictive Features and Responsiveness to Percutaneous Dilation.
Poupart S, Navarro-Castellanos I, Raboisson MJ, Lapierre C, Dery J, Miró J, Dahdah N
Pediatr Cardiol 2021 Apr;42(4):814-820. Epub 2021 Jan 19 doi: 10.1007/s00246-021-02545-w. PMID: 33464372
Transcatheter pulmonary valvuloplasty in neonates with pulmonary atresia and intact ventricular septum.
Hascoët S, Borrhomée S, Tahhan N, Petit J, Boet A, Houyel L, Lebret E, Ly M, Roussin R, Belli E, Lambert V, Laux D
Arch Cardiovasc Dis 2019 May;112(5):323-333. Epub 2019 Feb 20 doi: 10.1016/j.acvd.2018.11.015. PMID: 30797733

Recent systematic reviews

A Systematic Review of Benefits and Risks of Fetal Surgery for Congenital Cardiac Defects Such as Pulmonary Valve Stenosis and Critical Aortic Stenosis.
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
Fetal Echocardiographic Predictors of Postnatal Surgical Strategies in Critical Pulmonary Stenosis or Atresia with Intact Ventricular Septum: A Meta-Analysis.
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
Exposure to air pollutants and risk of congenital anomalies: A systematic review and metaanalysis.
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
Clinical performance of decellularized heart valves versus standard tissue conduits: a systematic review and meta-analysis.
Waqanivavalagi SWFR, Bhat S, Ground MB, Milsom PF, Cornish J
J Cardiothorac Surg 2020 Sep 18;15(1):260. doi: 10.1186/s13019-020-01292-y. PMID: 32948234Free PMC Article
Association between maternal body mass index and congenital heart defects in offspring: a systematic review.
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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    Curated

    • NICE, 2021
      UK NICE Guideline NG208, Heart valve disease presenting in adults: investigation and management, 2021

    Consumer resources

    Reviews

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