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Tetralogy of Fallot with absent pulmonary valve

MedGen UID:
488963
•Concept ID:
C1302264
•
Congenital Abnormality
Synonym: Tetralogy of Fallot with absent pulmonary valve syndrome
SNOMED CT: Tetralogy of Fallot with absent pulmonary valve (399228007)
 
HPO: HP:0011659

Definition

Features of tetralogy of Fallot with either rudimentary ridges or the complete absence of pulmonic valve tissue. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Tetralogy of Fallot with absent pulmonary valve

Professional guidelines

PubMed

Prenatal Diagnosis, Associations and Outcome for Fetuses with Congenital Absence of the Pulmonary Valve Syndrome.
Babaoğlu K, Doğan Y, Erdem S, Özbarlas N, Başar E, Uzun O
Anatol J Cardiol 2022 Sep;26(9):702-709. doi: 10.5152/AnatolJCardiol.2022.1461. PMID: 35943311Free PMC Article
Population-based review of tetralogy of Fallot with absent pulmonary valve: is prenatal diagnosis really associated with a poor prognosis?
Mivelaz Y, Lim KI, Templeton C, Campbell AI, Potts JE, Sandor GG
Ultrasound Obstet Gynecol 2012 Nov;40(5):536-41. doi: 10.1002/uog.11126. PMID: 22302386
Tetralogy of Fallot with absent pulmonary valve: diagnostic features and management.
Saxena KK, Joseph G, Thomas CS, John S, Krishnaswami S
Indian Heart J 1991 Nov-Dec;43(6):449-53. PMID: 1823893

Recent clinical studies

Etiology

Long Term Outcomes of Tetralogy of Fallot With Absent Pulmonary Valve (from the Pediatric Cardiac Care Consortium).
Siddeek H, Lunos S, Thomas AS, McCracken C, Steinberger J, Kochilas L
Am J Cardiol 2021 Nov 1;158:118-123. Epub 2021 Sep 10 doi: 10.1016/j.amjcard.2021.07.039. PMID: 34511183Free PMC Article
Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis.
Chelliah A, Moon-Grady AJ, Peyvandi S, Chiu JS, Bost JE, Schidlow D, Carroll SJ, Davey B, Divanovic A, Hornberger L, Howley LW, Kavanaugh-McHugh A, Kovalchin JP, Levasseur SM, Lindblade CL, Morris SA, Ngwezi D, Pruetz JD, Puchalski MD, Rychik J, Samai C, Tacy TA, Tworetzky W, Vernon MM, Yeh J, Donofrio MT
J Am Heart Assoc 2021 Jun 15;10(12):e019713. Epub 2021 Jun 8 doi: 10.1161/JAHA.120.019713. PMID: 34098741Free PMC Article
Evaluation of cardiovascular morphology and airway-related abnormalities in tetralogy of fallot with absent pulmonary valve syndrome on multidetector computed tomography angiography.
Verma M, Pandey NN, Ojha V, Jagia P, Kumar S, Kothari SS, Saxena A
J Card Surg 2021 Aug;36(8):2697-2704. Epub 2021 May 16 doi: 10.1111/jocs.15639. PMID: 33993521
Early Infant Symptomatic Patients With Tetralogy of Fallot With Absent Pulmonary Valve: Pulmonary Artery Management and Airway Stabilization.
Chow OS, Hoganson DM, Kaza AK, Chávez M, Altin FH, Marx GR, Friedman KG, Jennings RW, Baird CW
Ann Thorac Surg 2020 Nov;110(5):1644-1650. Epub 2020 Jun 29 doi: 10.1016/j.athoracsur.2020.05.058. PMID: 32615094
Prognosis in tetralogy of Fallot with absent pulmonary valve.
Kawazu Y, Inamura N, Ishii R, Terashima Y, Hamamichi Y, Kayatani F, Iwai S, Kawata H, Kishimoto H
Pediatr Int 2015 Apr;57(2):210-6. Epub 2014 Nov 25 doi: 10.1111/ped.12493. PMID: 25203222

Diagnosis

A Fetus with Tetralogy of Fallot with Absent Pulmonary Valve Syndrome.
Perez P, Panchangam C
Neoreviews 2023 Sep 1;24(9):e603-e606. doi: 10.1542/neo.24-9-e603. PMID: 37653085
Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis.
Chelliah A, Moon-Grady AJ, Peyvandi S, Chiu JS, Bost JE, Schidlow D, Carroll SJ, Davey B, Divanovic A, Hornberger L, Howley LW, Kavanaugh-McHugh A, Kovalchin JP, Levasseur SM, Lindblade CL, Morris SA, Ngwezi D, Pruetz JD, Puchalski MD, Rychik J, Samai C, Tacy TA, Tworetzky W, Vernon MM, Yeh J, Donofrio MT
J Am Heart Assoc 2021 Jun 15;10(12):e019713. Epub 2021 Jun 8 doi: 10.1161/JAHA.120.019713. PMID: 34098741Free PMC Article
Evaluation of cardiovascular morphology and airway-related abnormalities in tetralogy of fallot with absent pulmonary valve syndrome on multidetector computed tomography angiography.
Verma M, Pandey NN, Ojha V, Jagia P, Kumar S, Kothari SS, Saxena A
J Card Surg 2021 Aug;36(8):2697-2704. Epub 2021 May 16 doi: 10.1111/jocs.15639. PMID: 33993521
Prognosis in tetralogy of Fallot with absent pulmonary valve.
Kawazu Y, Inamura N, Ishii R, Terashima Y, Hamamichi Y, Kayatani F, Iwai S, Kawata H, Kishimoto H
Pediatr Int 2015 Apr;57(2):210-6. Epub 2014 Nov 25 doi: 10.1111/ped.12493. PMID: 25203222
Population-based review of tetralogy of Fallot with absent pulmonary valve: is prenatal diagnosis really associated with a poor prognosis?
Mivelaz Y, Lim KI, Templeton C, Campbell AI, Potts JE, Sandor GG
Ultrasound Obstet Gynecol 2012 Nov;40(5):536-41. doi: 10.1002/uog.11126. PMID: 22302386

Therapy

Surgical Repair of Tetralogy of Fallot With Absent Pulmonary Valve: Favorable Long-Term Results.
Dorobantu DM, Stoicescu C, Tulloh RM, Stoica SC
Semin Thorac Cardiovasc Surg 2019 Winter;31(4):847-849. Epub 2019 May 29 doi: 10.1053/j.semtcvs.2019.05.022. PMID: 31150827
Takotsubo cardiomyopathy after transcatheter Edwards Sapien pulmonary valve placement in a patient with tetralogy of Fallot.
Aregullin EO, Garg R, Berman D
Pediatr Cardiol 2013;34(8):1972-5. Epub 2012 Sep 28 doi: 10.1007/s00246-012-0517-z. PMID: 23052664

Prognosis

Tetralogy of Fallot and absent pulmonary valve syndrome.
Nuri H, Virgone A
Multimed Man Cardiothorac Surg 2022 Nov 8;2022 doi: 10.1510/mmcts.2022.071. PMID: 36345972
Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis.
Chelliah A, Moon-Grady AJ, Peyvandi S, Chiu JS, Bost JE, Schidlow D, Carroll SJ, Davey B, Divanovic A, Hornberger L, Howley LW, Kavanaugh-McHugh A, Kovalchin JP, Levasseur SM, Lindblade CL, Morris SA, Ngwezi D, Pruetz JD, Puchalski MD, Rychik J, Samai C, Tacy TA, Tworetzky W, Vernon MM, Yeh J, Donofrio MT
J Am Heart Assoc 2021 Jun 15;10(12):e019713. Epub 2021 Jun 8 doi: 10.1161/JAHA.120.019713. PMID: 34098741Free PMC Article
Tetralogy of Fallot With Absent Pulmonary Valve and Nonconfluent Pulmonary Arteries: A Management Conundrum.
Swaminathan S, Agarwal A, Infante JC, Rosenkranz E
World J Pediatr Congenit Heart Surg 2020 Jul;11(4):NP168-NP171. Epub 2018 Aug 27 doi: 10.1177/2150135118775661. PMID: 30148690
Prognosis in tetralogy of Fallot with absent pulmonary valve.
Kawazu Y, Inamura N, Ishii R, Terashima Y, Hamamichi Y, Kayatani F, Iwai S, Kawata H, Kishimoto H
Pediatr Int 2015 Apr;57(2):210-6. Epub 2014 Nov 25 doi: 10.1111/ped.12493. PMID: 25203222
Population-based review of tetralogy of Fallot with absent pulmonary valve: is prenatal diagnosis really associated with a poor prognosis?
Mivelaz Y, Lim KI, Templeton C, Campbell AI, Potts JE, Sandor GG
Ultrasound Obstet Gynecol 2012 Nov;40(5):536-41. doi: 10.1002/uog.11126. PMID: 22302386

Clinical prediction guides

Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis.
Chelliah A, Moon-Grady AJ, Peyvandi S, Chiu JS, Bost JE, Schidlow D, Carroll SJ, Davey B, Divanovic A, Hornberger L, Howley LW, Kavanaugh-McHugh A, Kovalchin JP, Levasseur SM, Lindblade CL, Morris SA, Ngwezi D, Pruetz JD, Puchalski MD, Rychik J, Samai C, Tacy TA, Tworetzky W, Vernon MM, Yeh J, Donofrio MT
J Am Heart Assoc 2021 Jun 15;10(12):e019713. Epub 2021 Jun 8 doi: 10.1161/JAHA.120.019713. PMID: 34098741Free PMC Article
Evaluation of cardiovascular morphology and airway-related abnormalities in tetralogy of fallot with absent pulmonary valve syndrome on multidetector computed tomography angiography.
Verma M, Pandey NN, Ojha V, Jagia P, Kumar S, Kothari SS, Saxena A
J Card Surg 2021 Aug;36(8):2697-2704. Epub 2021 May 16 doi: 10.1111/jocs.15639. PMID: 33993521
Early Infant Symptomatic Patients With Tetralogy of Fallot With Absent Pulmonary Valve: Pulmonary Artery Management and Airway Stabilization.
Chow OS, Hoganson DM, Kaza AK, Chávez M, Altin FH, Marx GR, Friedman KG, Jennings RW, Baird CW
Ann Thorac Surg 2020 Nov;110(5):1644-1650. Epub 2020 Jun 29 doi: 10.1016/j.athoracsur.2020.05.058. PMID: 32615094
Prognosis in tetralogy of Fallot with absent pulmonary valve.
Kawazu Y, Inamura N, Ishii R, Terashima Y, Hamamichi Y, Kayatani F, Iwai S, Kawata H, Kishimoto H
Pediatr Int 2015 Apr;57(2):210-6. Epub 2014 Nov 25 doi: 10.1111/ped.12493. PMID: 25203222
Value of clinical and echocardiographic features in predicting outcome in the fetus, infant, and child with tetralogy of Fallot with absent pulmonary valve complex.
Moon-Grady AJ, Tacy TA, Brook MM, Hanley FL, Silverman NH
Am J Cardiol 2002 Jun 1;89(11):1280-5. doi: 10.1016/s0002-9149(02)02326-3. PMID: 12031728

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