U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Congenital pulmonary insufficiency

MedGen UID:
539526
•Concept ID:
C0265833
•
Congenital Abnormality
Synonyms: Congenital insufficiency of pulmonary valve; Congenital insufficiency of the pulmonary valve; Congenital pulmonary regurgitation; Congenital Pulmonary Valve Insufficiency
SNOMED CT: Congenital insufficiency of the pulmonary valve (15096009); Congenital insufficiency of pulmonary valve (15096009); Congenital pulmonary regurgitation (15096009)

Definition

Dysfunction of the pulmonary valve characterized by incomplete valve closure that is present at birth. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital pulmonary insufficiency

Professional guidelines

PubMed

Infant of a diabetic mother: clinical presentation, diagnosis and treatment.
Suda-Całus M, Dąbrowska K, Gulczyńska E
Pediatr Endocrinol Diabetes Metab 2024;30(1):36-41. doi: 10.5114/pedm.2024.137891. PMID: 39026477Free PMC Article
Diagnosis and treatment of congenital tuberculosis: a systematic review of 92 cases.
Li C, Liu L, Tao Y
Orphanet J Rare Dis 2019 Jun 10;14(1):131. doi: 10.1186/s13023-019-1101-x. PMID: 31182120Free PMC Article
Prenatal diagnosis and management of congenital diaphragmatic hernia.
Basurto D, Russo FM, Van der Veeken L, Van der Merwe J, Hooper S, Benachi A, De Bie F, Gomez O, Deprest J
Best Pract Res Clin Obstet Gynaecol 2019 Jul;58:93-106. Epub 2019 Jan 5 doi: 10.1016/j.bpobgyn.2018.12.010. PMID: 30772144

Recent clinical studies

Etiology

Outcomes of transcatheter pulmonary SAPIEN 3 valve implantation: an international registry.
Hascoët S, Bentham JR, Giugno L, Betrián-Blasco P, Kempny A, Houeijeh A, Baho H, Sharma SR, Jones MI, Biernacka EK, Combes N, Georgiev S, Bouvaist H, Martins JD, Kantzis M, Turner M, Schubert S, Jalal Z, Butera G, Malekzadeh-Milani S, Valdeolmillos E, Karsenty C, Ödemiş E, Aldebert P, Haas NA, Khatib I, Wåhlander H, Gaio G, Mendoza A, Arif S, Castaldi B, Dohlen G, Carere RG, Del Cerro-Marin MJ, Kitzmüller E, Hermuzi A, Carminati M, Guérin P, Tengler A, Fraisse A; EUROPULMS3 investigators
Eur Heart J 2024 Jan 14;45(3):198-210. doi: 10.1093/eurheartj/ehad663. PMID: 37874971
Unmet Clinical Needs for Transcatheter Pulmonary Valves.
Agwu N, Recto MR, Kheradvar A
Ann Biomed Eng 2023 Nov;51(11):2384-2392. Epub 2023 Aug 5 doi: 10.1007/s10439-023-03328-5. PMID: 37543538Free PMC Article
Pulmonary Valve Replacement in Tetralogy of Fallot: An Updated Meta-Analysis.
Van den Eynde J, Sá MPBO, Vervoort D, Roever L, Meyns B, Budts W, Gewillig M, Ruhparwar A, Zhigalov K, Weymann A
Ann Thorac Surg 2022 Mar;113(3):1036-1046. Epub 2020 Dec 27 doi: 10.1016/j.athoracsur.2020.11.040. PMID: 33378694
Imaging of the right heart--CT and CMR.
Kochav J, Simprini L, Weinsaft JW
Echocardiography 2015 Jan;32 Suppl 1:S53-68. Epub 2014 Sep 19 doi: 10.1111/echo.12212. PMID: 25244072
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

Valvular Heart Disease.
Kisling A, Gallagher R
Prim Care 2024 Mar;51(1):95-109. Epub 2023 Sep 7 doi: 10.1016/j.pop.2023.08.003. PMID: 38278576
Current outcomes and treatment of tetralogy of Fallot.
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
Congenital pulmonary lymphangiectasia.
Yuan SM
J Perinat Med 2017 Dec 20;45(9):1023-1030. doi: 10.1515/jpm-2016-0407. PMID: 28258976
Ventricular septal defect.
Penny DJ, Vick GW 3rd
Lancet 2011 Mar 26;377(9771):1103-12. Epub 2011 Feb 23 doi: 10.1016/S0140-6736(10)61339-6. PMID: 21349577
Tetralogy of Fallot.
Apitz C, Webb GD, Redington AN
Lancet 2009 Oct 24;374(9699):1462-71. Epub 2009 Aug 14 doi: 10.1016/S0140-6736(09)60657-7. PMID: 19683809

Therapy

Pulmonary Valve Replacement in Tetralogy of Fallot: An Updated Meta-Analysis.
Van den Eynde J, Sá MPBO, Vervoort D, Roever L, Meyns B, Budts W, Gewillig M, Ruhparwar A, Zhigalov K, Weymann A
Ann Thorac Surg 2022 Mar;113(3):1036-1046. Epub 2020 Dec 27 doi: 10.1016/j.athoracsur.2020.11.040. PMID: 33378694
Extracorporeal Membrane Oxygenation for Hemodynamic Support.
Straube T, Cheifetz IM, Jackson KW
Clin Perinatol 2020 Sep;47(3):671-684. Epub 2020 May 26 doi: 10.1016/j.clp.2020.05.016. PMID: 32713457
The Natural History of Early-onset Scoliosis.
Karol LA
J Pediatr Orthop 2019 Jul;39(Issue 6, Supplement 1 Suppl 1):S38-S43. doi: 10.1097/BPO.0000000000001351. PMID: 31169646
Home ventilation.
Simonds AK
Eur Respir J Suppl 2003 Nov;47:38s-46s. doi: 10.1183/09031936.03.00029803. PMID: 14621116
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

Diagnosis of major heart defects by routine first-trimester ultrasound examination: association with increased nuchal translucency, tricuspid regurgitation and abnormal flow in ductus venosus.
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
Current outcomes and treatment of tetralogy of Fallot.
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
Congenital pulmonary lymphangiectasia.
Yuan SM
J Perinat Med 2017 Dec 20;45(9):1023-1030. doi: 10.1515/jpm-2016-0407. PMID: 28258976
Tetralogy of Fallot.
Apitz C, Webb GD, Redington AN
Lancet 2009 Oct 24;374(9699):1462-71. Epub 2009 Aug 14 doi: 10.1016/S0140-6736(09)60657-7. PMID: 19683809
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

Clinical prediction guides

Outcomes of transcatheter pulmonary SAPIEN 3 valve implantation: an international registry.
Hascoët S, Bentham JR, Giugno L, Betrián-Blasco P, Kempny A, Houeijeh A, Baho H, Sharma SR, Jones MI, Biernacka EK, Combes N, Georgiev S, Bouvaist H, Martins JD, Kantzis M, Turner M, Schubert S, Jalal Z, Butera G, Malekzadeh-Milani S, Valdeolmillos E, Karsenty C, Ödemiş E, Aldebert P, Haas NA, Khatib I, Wåhlander H, Gaio G, Mendoza A, Arif S, Castaldi B, Dohlen G, Carere RG, Del Cerro-Marin MJ, Kitzmüller E, Hermuzi A, Carminati M, Guérin P, Tengler A, Fraisse A; EUROPULMS3 investigators
Eur Heart J 2024 Jan 14;45(3):198-210. doi: 10.1093/eurheartj/ehad663. PMID: 37874971
Outcomes After Hybrid Palliation for Infants With Critical Left Heart Obstruction.
Argo MB, Barron DJ, Eghtesady P, Yerebakan C, DeCampli WM, Alsoufi B, Honjo O, Jacobs JP, Paramananthan T, Rahman M, Lambert LM, Jegatheeswaran A, Carrillo SA, Husain SA, Ramakrishnan K, Caldarone CA, Karamlou T, Nelson J, Mannie C, Romano JC, Turek JW, Blackstone EH, Galantowicz ME, Kirklin JK, Mitchell ME, McCrindle BW; Congenital Heart Surgeons Society Critical Left Heart Obstruction Work Group
J Am Coll Cardiol 2023 Oct 3;82(14):1427-1441. doi: 10.1016/j.jacc.2023.07.020. PMID: 37758438
Reexamining remodelling in children.
Matsushima S, Heß A, Lämmerzahl JR, Karliova I, Giebels C, Schäfers HJ
Eur J Cardiothorac Surg 2020 Jun 1;57(6):1091-1097. doi: 10.1093/ejcts/ezz380. PMID: 31972004
Diagnosis of major heart defects by routine first-trimester ultrasound examination: association with increased nuchal translucency, tricuspid regurgitation and abnormal flow in ductus venosus.
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
Current outcomes and treatment of tetralogy of Fallot.
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

Recent systematic reviews

Association between immediate oxygenation response and survival in preterm infants receiving rescue inhaled nitric oxide therapy for hypoxemia from pulmonary hypertension: A systematic review and meta-analysis.
Baczynski M, Jasani B, De Castro C, Dani C, Subhedar NV, Chandrasekharan P, Lakshminrusimha S, McNamara PJ, Bischoff AR, Jain A
Early Hum Dev 2023 Sep;184:105841. Epub 2023 Aug 2 doi: 10.1016/j.earlhumdev.2023.105841. PMID: 37542786
Outcomes of truncal valve replacement in neonates and infants: a meta-analysis.
Hardy WA, Kang L, Turek JW, Rajab TK
Cardiol Young 2023 May;33(5):673-680. Epub 2023 Mar 27 doi: 10.1017/S1047951123000604. PMID: 36970855
End-Diastolic Forward Flow and Restrictive Physiology in Repaired Tetralogy of Fallot: A Systematic Review and Meta-Analysis.
Van den Eynde J, Derdeyn E, Schuermans A, Shivaram P, Budts W, Danford DA, Kutty S
J Am Heart Assoc 2022 Apr 5;11(7):e024036. Epub 2022 Mar 18 doi: 10.1161/JAHA.121.024036. PMID: 35301867Free PMC Article
Pulmonary Valve Replacement in Tetralogy of Fallot: An Updated Meta-Analysis.
Van den Eynde J, Sá MPBO, Vervoort D, Roever L, Meyns B, Budts W, Gewillig M, Ruhparwar A, Zhigalov K, Weymann A
Ann Thorac Surg 2022 Mar;113(3):1036-1046. Epub 2020 Dec 27 doi: 10.1016/j.athoracsur.2020.11.040. PMID: 33378694
Diagnosis and treatment of congenital tuberculosis: a systematic review of 92 cases.
Li C, Liu L, Tao Y
Orphanet J Rare Dis 2019 Jun 10;14(1):131. doi: 10.1186/s13023-019-1101-x. PMID: 31182120Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...