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Unguarded tricuspid valve

MedGen UID:
576468
•Concept ID:
C0344745
•
Congenital Abnormality
Synonyms: Absent tricuspid leaflet; Rudimentary tricuspid valve leaflets; Unguarded tricuspid valve orifice
SNOMED CT: Absent tricuspid leaflet (253381001); Unguarded tricuspid valve orifice (253381001)
 
HPO: HP:0030719

Definition

A form of agenesis of the tricuspid valve in which (although the normal orifice between the right atrium and right ventricle exists) there is no tricuspid valvular tissue. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUnguarded tricuspid valve

Recent clinical studies

Etiology

Surgical management of congenital (non-Ebstein) tricuspid valve regurgitation.
Said SM, Burkhart HM, Dearani JA
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2012;15(1):46-60. doi: 10.1053/j.pcsu.2012.01.009. PMID: 22424508
Echocardiographic spectrum of congenitally unguarded tricuspid valve orifice and patent right ventricular outflow tract.
Mohan JC, Passey R, Arora R
Int J Cardiol 2000 Jul 31;74(2-3):153-7. doi: 10.1016/s0167-5273(00)00263-1. PMID: 10962115
First-trimester diagnosis of fetal congenital heart disease by transvaginal ultrasonography.
Achiron R, Rotstein Z, Lipitz S, Mashiach S, Hegesh J
Obstet Gynecol 1994 Jul;84(1):69-72. PMID: 8008327
Tricuspid valve disease with significant tricuspid insufficiency in the fetus: diagnosis and outcome.
Hornberger LK, Sahn DJ, Kleinman CS, Copel JA, Reed KL
J Am Coll Cardiol 1991 Jan;17(1):167-73. doi: 10.1016/0735-1097(91)90722-l. PMID: 1987222

Diagnosis

Diagnosis of fetal congenitally unguarded tricuspid valve orifice by echocardiography.
Liu H, Yuan G, Li X, Song Y, Wang C, Zhang C
Echocardiography 2022 Oct;39(10):1324-1327. Epub 2022 Oct 2 doi: 10.1111/echo.15457. PMID: 36184263
Left-sided congenitally unguarded tricuspid valve with congenitally corrected transposition of the great arteries: A rare diagnosis confirmed by three-dimensional echocardiography.
Williams JL, Lockhart JL, Miller SG, Barker PCA, Campbell MJ
Echocardiography 2020 Jul;37(7):1101-1104. Epub 2020 Jul 11 doi: 10.1111/echo.14683. PMID: 32652546
A case of unguarded tricuspid valve orifice diagnosed accidentally in an adult.
Abdelnabi MH, Shehata H, Saleh Y, Elgowelly M, Almaghraby A
Echocardiography 2019 Mar;36(3):615-617. Epub 2019 Feb 9 doi: 10.1111/echo.14273. PMID: 30738004
Color Doppler flow pattern in antenatal diagnosis of unguarded tricuspid valve.
Indrani S, Vijayalakshmi R, Suresh S
Ultrasound Obstet Gynecol 2005 May;25(5):514-6. doi: 10.1002/uog.1728. PMID: 15704242
Tricuspid valve disease with significant tricuspid insufficiency in the fetus: diagnosis and outcome.
Hornberger LK, Sahn DJ, Kleinman CS, Copel JA, Reed KL
J Am Coll Cardiol 1991 Jan;17(1):167-73. doi: 10.1016/0735-1097(91)90722-l. PMID: 1987222

Therapy

A case of unguarded tricuspid valve orifice diagnosed accidentally in an adult.
Abdelnabi MH, Shehata H, Saleh Y, Elgowelly M, Almaghraby A
Echocardiography 2019 Mar;36(3):615-617. Epub 2019 Feb 9 doi: 10.1111/echo.14273. PMID: 30738004

Prognosis

Prediction of outcome of tricuspid valve malformations diagnosed during fetal life.
Andrews RE, Tibby SM, Sharland GK, Simpson JM
Am J Cardiol 2008 Apr 1;101(7):1046-50. Epub 2008 Feb 6 doi: 10.1016/j.amjcard.2007.11.049. PMID: 18359329
Echocardiographic spectrum of congenitally unguarded tricuspid valve orifice and patent right ventricular outflow tract.
Mohan JC, Passey R, Arora R
Int J Cardiol 2000 Jul 31;74(2-3):153-7. doi: 10.1016/s0167-5273(00)00263-1. PMID: 10962115
Tricuspid valve disease with significant tricuspid insufficiency in the fetus: diagnosis and outcome.
Hornberger LK, Sahn DJ, Kleinman CS, Copel JA, Reed KL
J Am Coll Cardiol 1991 Jan;17(1):167-73. doi: 10.1016/0735-1097(91)90722-l. PMID: 1987222

Clinical prediction guides

Prediction of outcome of tricuspid valve malformations diagnosed during fetal life.
Andrews RE, Tibby SM, Sharland GK, Simpson JM
Am J Cardiol 2008 Apr 1;101(7):1046-50. Epub 2008 Feb 6 doi: 10.1016/j.amjcard.2007.11.049. PMID: 18359329

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