U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

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
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
Mid-term results of correction of Tetralogy of Fallot with absent pulmonary valve.
Talwar S, Divya A, Choudhary SK, Gupta SK, Ramakriahnan S, Kothari SS, Juneja R, Saxena A, Airan B
Indian Heart J 2017 Nov-Dec;69(6):767-771. Epub 2017 Apr 18 doi: 10.1016/j.ihj.2017.04.009. PMID: 29174256Free PMC Article
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
Tetralogy of fallot with absent pulmonary valve: simplified technique for homograft repair.
Kirshbom PM, Jaggers JJ, Ungerleider RM
J Thorac Cardiovasc Surg 1999 Dec;118(6):1125-7. doi: 10.1016/S0022-5223(99)70116-9. PMID: 10595993

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
Tetralogy of Fallot with absent pulmonary valve: main differences with classic Fallot are crucial for an accurate prenatal diagnosis and counselling.
Ataíde Silva R, Martins D, Teixeira A, Anjos R
BMJ Case Rep 2020 Apr 9;13(4) doi: 10.1136/bcr-2019-233592. PMID: 32276999Free PMC Article
Combination of tetralogy of Fallot with absent pulmonary valve and left pulmonary artery originating from patent ductus arteriosus: A rare association in an infant.
Saygı M, Kılınç F, İrdem A, Öztürk E, Güzeltaş A
Anatol J Cardiol 2015 May 1;15(5):E15-6. doi: 10.5152/akd.2015.6148. PMID: 25993731Free PMC Article
Tetralogy of fallot with absent pulmonary valve: imaging with today's diagnostic tools.
Ugurlucan M, Arslan AH, Yildiz Y, Besikci RT, Karadeniz O, Cicek S
World J Pediatr Congenit Heart Surg 2013 Jul;4(3):322-3. doi: 10.1177/2150135113478638. PMID: 24327508
Tetralogy of Fallot with absent pulmonary valve. Natural history and hemodynamic considerations.
Lakier JB, Stanger P, Heymann MA, Hoffman JI, Rudolph AM
Circulation 1974 Jul;50(1):167-75. doi: 10.1161/01.cir.50.1.167. PMID: 4835262

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
Tetralogy of Fallot with absent pulmonary valve: main differences with classic Fallot are crucial for an accurate prenatal diagnosis and counselling.
Ataíde Silva R, Martins D, Teixeira A, Anjos R
BMJ Case Rep 2020 Apr 9;13(4) doi: 10.1136/bcr-2019-233592. PMID: 32276999Free PMC Article
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
Tetralogy of fallot with absent pulmonary valve: simplified technique for homograft repair.
Kirshbom PM, Jaggers JJ, Ungerleider RM
J Thorac Cardiovasc Surg 1999 Dec;118(6):1125-7. doi: 10.1016/S0022-5223(99)70116-9. PMID: 10595993
Tetralogy of Fallot with absent pulmonary valve. Natural history and hemodynamic considerations.
Lakier JB, Stanger P, Heymann MA, Hoffman JI, Rudolph AM
Circulation 1974 Jul;50(1):167-75. doi: 10.1161/01.cir.50.1.167. PMID: 4835262

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
Surgical outcome after complete repair of tetralogy of Fallot with absent pulmonary valve: comparison between bovine jugular vein-valved conduit and monocusp-valve patch.
Wang ES, Fan XS, Xiang L, Li SJ, Zhang H
World J Pediatr 2018 Oct;14(5):510-519. Epub 2018 Jul 30 doi: 10.1007/s12519-018-0169-z. PMID: 30062647
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

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.

    Consumer resources

    Reviews

    Related information

    • ClinVar
      Related medical variations
    • 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...