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Abnormal pulmonary valve physiology

MedGen UID:
1640121
•Concept ID:
C4703466
•
Pathologic Function
HPO: HP:0031654

Definition

Any functional anomaly of the pumonary valve. [from HPO]

Professional guidelines

PubMed

The American Association for Thoracic Surgery (AATS) 2024 expert consensus document: Management of neonates and infants with Ebstein anomaly.
Konstantinov IE, Chai P, Bacha E, Caldarone CA, Da Silva JP, Da Fonseca Da Silva L, Dearani J, Hornberger L, Knott-Craig C, Del Nido P, Qureshi M, Sarris G, Starnes V, Tsang V
J Thorac Cardiovasc Surg 2024 Aug;168(2):311-324. Epub 2024 Apr 27 doi: 10.1016/j.jtcvs.2024.04.018. PMID: 38685467
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
SOS1 mutations in Noonan syndrome: molecular spectrum, structural insights on pathogenic effects, and genotype-phenotype correlations.
Lepri F, De Luca A, Stella L, Rossi C, Baldassarre G, Pantaleoni F, Cordeddu V, Williams BJ, Dentici ML, Caputo V, Venanzi S, Bonaguro M, Kavamura I, Faienza MF, Pilotta A, Stanzial F, Faravelli F, Gabrielli O, Marino B, Neri G, Silengo MC, Ferrero GB, Torrrente I, Selicorni A, Mazzanti L, Digilio MC, Zampino G, Dallapiccola B, Gelb BD, Tartaglia M
Hum Mutat 2011 Jul;32(7):760-72. Epub 2011 Apr 28 doi: 10.1002/humu.21492. PMID: 21387466Free PMC Article

Recent clinical studies

Etiology

Management and outcome of prenatal absent pulmonary valve syndrome.
Recker F, Weber EC, Strizek B, Geipel A, Berg C, Gembruch U
Arch Gynecol Obstet 2022 Nov;306(5):1449-1454. Epub 2022 Jan 18 doi: 10.1007/s00404-022-06397-4. PMID: 35043273Free PMC Article
Fetal cardiac interventions: Where do we stand?
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
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
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
Percutaneous pulmonary valve endocarditis: incidence, prevention and management.
Patel M, Malekzadeh-Milani S, Ladouceur M, Iserin L, Boudjemline Y
Arch Cardiovasc Dis 2014 Nov;107(11):615-24. Epub 2014 Oct 16 doi: 10.1016/j.acvd.2014.07.052. PMID: 25445753

Diagnosis

The heart in RASopathies.
Delogu AB, Limongelli G, Versacci P, Adorisio R, Kaski JP, Blandino R, Maiolo S, Monda E, Putotto C, De Rosa G, Chatfield KC, Gelb BD, Calcagni G
Am J Med Genet C Semin Med Genet 2022 Dec;190(4):440-451. Epub 2022 Nov 21 doi: 10.1002/ajmg.c.32014. PMID: 36408797
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
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
What is aortic overriding?
Anderson RH, Spicer DE, Henry GW, Rigsby C, Hlavacek AM, Mohun TJ
Cardiol Young 2015 Apr;25(4):612-25. Epub 2014 Jul 1 doi: 10.1017/S1047951114001139. PMID: 24983250
LEOPARD syndrome.
Massoure PL, Latremouille C, Lamblin G, Leca F
Cardiovasc J Afr 2012 Jul 12;23(6):e19-20. doi: 10.5830/CVJA-2012-011. PMID: 22832481

Therapy

The American Association for Thoracic Surgery (AATS) 2024 expert consensus document: Management of neonates and infants with Ebstein anomaly.
Konstantinov IE, Chai P, Bacha E, Caldarone CA, Da Silva JP, Da Fonseca Da Silva L, Dearani J, Hornberger L, Knott-Craig C, Del Nido P, Qureshi M, Sarris G, Starnes V, Tsang V
J Thorac Cardiovasc Surg 2024 Aug;168(2):311-324. Epub 2024 Apr 27 doi: 10.1016/j.jtcvs.2024.04.018. PMID: 38685467
Tetralogy of Fallot.
Krieger EV, Valente AM
Cardiol Clin 2020 Aug;38(3):365-377. Epub 2020 Jun 10 doi: 10.1016/j.ccl.2020.04.009. PMID: 32622491
Fetal cardiac interventions: Where do we stand?
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
Percutaneous pulmonary valve endocarditis: incidence, prevention and management.
Patel M, Malekzadeh-Milani S, Ladouceur M, Iserin L, Boudjemline Y
Arch Cardiovasc Dis 2014 Nov;107(11):615-24. Epub 2014 Oct 16 doi: 10.1016/j.acvd.2014.07.052. PMID: 25445753
Pediatric cardiac interventions.
Hijazi ZM, Awad SM
JACC Cardiovasc Interv 2008 Dec;1(6):603-11. doi: 10.1016/j.jcin.2008.07.007. PMID: 19463373

Prognosis

Congenital central pulmonary artery anomalies: Part 2.
Newman B, Alkhori N
Pediatr Radiol 2020 Jul;50(8):1030-1040. Epub 2020 Jun 4 doi: 10.1007/s00247-020-04703-3. PMID: 32500159
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
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
Percutaneous pulmonary valve endocarditis: incidence, prevention and management.
Patel M, Malekzadeh-Milani S, Ladouceur M, Iserin L, Boudjemline Y
Arch Cardiovasc Dis 2014 Nov;107(11):615-24. Epub 2014 Oct 16 doi: 10.1016/j.acvd.2014.07.052. PMID: 25445753
Congenitally corrected transposition.
Wallis GA, Debich-Spicer D, Anderson RH
Orphanet J Rare Dis 2011 May 14;6:22. doi: 10.1186/1750-1172-6-22. PMID: 21569592Free PMC Article

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
Management and outcome of prenatal absent pulmonary valve syndrome.
Recker F, Weber EC, Strizek B, Geipel A, Berg C, Gembruch U
Arch Gynecol Obstet 2022 Nov;306(5):1449-1454. Epub 2022 Jan 18 doi: 10.1007/s00404-022-06397-4. PMID: 35043273Free PMC Article
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
Tricuspid Valve Imaging and Intervention in Pediatric and Adult Patients With Congenital Heart Disease.
Qureshi MY, Sommer RJ, Cabalka AK
JACC Cardiovasc Imaging 2019 Apr;12(4):637-651. doi: 10.1016/j.jcmg.2018.10.036. PMID: 30947906
LEOPARD syndrome.
Massoure PL, Latremouille C, Lamblin G, Leca F
Cardiovasc J Afr 2012 Jul 12;23(6):e19-20. doi: 10.5830/CVJA-2012-011. PMID: 22832481

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
Absent pulmonary valve with tricuspid atresia/stenosis: literature review with new three long-term cases.
Kawasaki Y, Murakami Y, Fujino M, Sasaki T, Nakamura K, Yoshida Y, Suzuki T, Nishigaki K, Ehara E
Heart Vessels 2022 Jan;37(1):142-151. Epub 2021 Jun 5 doi: 10.1007/s00380-021-01887-y. PMID: 34089363
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
Biventricular repair versus uni-ventricular repair for pulmonary atresia with intact ventrical septum: A systematic review.
Li FF, Du XL, Chen S
J Huazhong Univ Sci Technolog Med Sci 2015 Oct;35(5):656-661. Epub 2015 Oct 22 doi: 10.1007/s11596-015-1485-3. PMID: 26489617
Cardiorespiratory Response to Exercise before and after Pulmonary Valve Replacement in Patients with Repaired Tetralogy of Fallot: A Retrospective Study and Systematic Review of the Literature.
Sabate Rotes A, Johnson JN, Burkhart HM, Eidem BW, Allison TG, Driscoll DJ
Congenit Heart Dis 2015 May-Jun;10(3):263-70. Epub 2014 Aug 4 doi: 10.1111/chd.12207. PMID: 25088332

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