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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

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
Fetal neck tumors - antenatal and intrapartum management.
Kornacki J, Skrzypczak J
Ginekol Pol 2017;88(5):266-269. doi: 10.5603/GP.a2017.0050. PMID: 28580573

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
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
Pulmonary valve replacement after repaired Tetralogy of Fallot.
Tatewaki H, Shiose A
Gen Thorac Cardiovasc Surg 2018 Sep;66(9):509-515. Epub 2018 May 19 doi: 10.1007/s11748-018-0931-0. PMID: 29779123
Pulmonary regurgitation: not a benign lesion.
Bouzas B, Kilner PJ, Gatzoulis MA
Eur Heart J 2005 Mar;26(5):433-9. Epub 2005 Jan 7 doi: 10.1093/eurheartj/ehi091. PMID: 15640261
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
Oxygen and pulmonary vasodilation: The role of oxidative and nitrosative stress.
Steinhorn RH, Lakshminrusimha S
Semin Fetal Neonatal Med 2020 Apr;25(2):101083. Epub 2020 Jan 16 doi: 10.1016/j.siny.2020.101083. PMID: 31983672
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
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
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
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
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 systematic reviews

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
Tricuspid Valve Intervention at the Time of Pulmonary Valve Replacement in Adults With Congenital Heart Disease: A Systematic Review and Meta-Analysis.
Van den Eynde J, Callahan CP, Lo Rito M, Hussein N, Carvajal H, Guariento A, Ruhparwar A, Weymann A, Budts W, Gewillig M, Sá MP, Kutty S
J Am Heart Assoc 2021 Dec 21;10(24):e022909. Epub 2021 Dec 7 doi: 10.1161/JAHA.121.022909. PMID: 34873914Free 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
Nitric oxide for respiratory failure in infants born at or near term.
Barrington KJ, Finer N, Pennaforte T, Altit G
Cochrane Database Syst Rev 2017 Jan 5;1(1):CD000399. doi: 10.1002/14651858.CD000399.pub3. PMID: 28056166Free PMC Article

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