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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

Prenatal Diagnosis and Management of Single-Ventricle Heart Disease.
Freud LR, Seed M
Can J Cardiol 2022 Jul;38(7):897-908. Epub 2022 Apr 13 doi: 10.1016/j.cjca.2022.04.003. PMID: 35429589
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
Management of the adult Fontan patient.
Rodriguez FH, Book WM
Heart 2020 Jan;106(2):105-110. Epub 2019 Oct 29 doi: 10.1136/heartjnl-2019-314937. PMID: 31662332

Recent clinical studies

Etiology

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
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
Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study.
Freud LR, Escobar-Diaz MC, Kalish BT, Komarlu R, Puchalski MD, Jaeggi ET, Szwast AL, Freire G, Levasseur SM, Kavanaugh-McHugh A, Michelfelder EC, Moon-Grady AJ, Donofrio MT, Howley LW, Tierney ES, Cuneo BF, Morris SA, Pruetz JD, van der Velde ME, Kovalchin JP, Ikemba CM, Vernon MM, Samai C, Satou GM, Gotteiner NL, Phoon CK, Silverman NH, McElhinney DB, Tworetzky W
Circulation 2015 Aug 11;132(6):481-9. Epub 2015 Jun 9 doi: 10.1161/CIRCULATIONAHA.115.015839. PMID: 26059011Free PMC Article

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
Pulmonary Atresia With an Intact Ventricular Septum: Preoperative Physiology, Imaging, and Management.
Chikkabyrappa SM, Loomba RS, Tretter JT
Semin Cardiothorac Vasc Anesth 2018 Sep;22(3):245-255. Epub 2018 Feb 7 doi: 10.1177/1089253218756757. PMID: 29411679
Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study.
Freud LR, Escobar-Diaz MC, Kalish BT, Komarlu R, Puchalski MD, Jaeggi ET, Szwast AL, Freire G, Levasseur SM, Kavanaugh-McHugh A, Michelfelder EC, Moon-Grady AJ, Donofrio MT, Howley LW, Tierney ES, Cuneo BF, Morris SA, Pruetz JD, van der Velde ME, Kovalchin JP, Ikemba CM, Vernon MM, Samai C, Satou GM, Gotteiner NL, Phoon CK, Silverman NH, McElhinney DB, Tworetzky W
Circulation 2015 Aug 11;132(6):481-9. Epub 2015 Jun 9 doi: 10.1161/CIRCULATIONAHA.115.015839. PMID: 26059011Free PMC Article

Therapy

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
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
Tetralogy of Fallot in the Current Era.
Mouws EMJP, de Groot NMS, van de Woestijne PC, de Jong PL, Helbing WA, van Beynum IM, Bogers AJJC
Semin Thorac Cardiovasc Surg 2019 Autumn;31(3):496-504. Epub 2018 Nov 2 doi: 10.1053/j.semtcvs.2018.10.015. PMID: 30395964
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

Prognosis

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
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
Pulmonary Atresia With an Intact Ventricular Septum: Preoperative Physiology, Imaging, and Management.
Chikkabyrappa SM, Loomba RS, Tretter JT
Semin Cardiothorac Vasc Anesth 2018 Sep;22(3):245-255. Epub 2018 Feb 7 doi: 10.1177/1089253218756757. PMID: 29411679
Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study.
Freud LR, Escobar-Diaz MC, Kalish BT, Komarlu R, Puchalski MD, Jaeggi ET, Szwast AL, Freire G, Levasseur SM, Kavanaugh-McHugh A, Michelfelder EC, Moon-Grady AJ, Donofrio MT, Howley LW, Tierney ES, Cuneo BF, Morris SA, Pruetz JD, van der Velde ME, Kovalchin JP, Ikemba CM, Vernon MM, Samai C, Satou GM, Gotteiner NL, Phoon CK, Silverman NH, McElhinney DB, Tworetzky W
Circulation 2015 Aug 11;132(6):481-9. Epub 2015 Jun 9 doi: 10.1161/CIRCULATIONAHA.115.015839. PMID: 26059011Free PMC Article

Clinical prediction guides

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
Tetralogy of Fallot in the Current Era.
Mouws EMJP, de Groot NMS, van de Woestijne PC, de Jong PL, Helbing WA, van Beynum IM, Bogers AJJC
Semin Thorac Cardiovasc Surg 2019 Autumn;31(3):496-504. Epub 2018 Nov 2 doi: 10.1053/j.semtcvs.2018.10.015. PMID: 30395964
Pulmonary Atresia With an Intact Ventricular Septum: Preoperative Physiology, Imaging, and Management.
Chikkabyrappa SM, Loomba RS, Tretter JT
Semin Cardiothorac Vasc Anesth 2018 Sep;22(3):245-255. Epub 2018 Feb 7 doi: 10.1177/1089253218756757. PMID: 29411679
Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study.
Freud LR, Escobar-Diaz MC, Kalish BT, Komarlu R, Puchalski MD, Jaeggi ET, Szwast AL, Freire G, Levasseur SM, Kavanaugh-McHugh A, Michelfelder EC, Moon-Grady AJ, Donofrio MT, Howley LW, Tierney ES, Cuneo BF, Morris SA, Pruetz JD, van der Velde ME, Kovalchin JP, Ikemba CM, Vernon MM, Samai C, Satou GM, Gotteiner NL, Phoon CK, Silverman NH, McElhinney DB, Tworetzky W
Circulation 2015 Aug 11;132(6):481-9. Epub 2015 Jun 9 doi: 10.1161/CIRCULATIONAHA.115.015839. PMID: 26059011Free PMC Article

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
The Ross Procedure: A Systematic Review, Meta-Analysis, and Microsimulation.
Etnel JRG, Grashuis P, Huygens SA, Pekbay B, Papageorgiou G, Helbing WA, Roos-Hesselink JW, Bogers AJJC, Mokhles MM, Takkenberg JJM
Circ Cardiovasc Qual Outcomes 2018 Dec;11(12):e004748. doi: 10.1161/CIRCOUTCOMES.118.004748. PMID: 30562065
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
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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