U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Tricuspid atresia

MedGen UID:
67034
Concept ID:
C0243002
Congenital Abnormality
Synonyms: Congenital agenesis of the tricuspid valve; Tricuspid atresia (disease)
SNOMED CT: Congenital atresia of tricuspid valve (63042009); Tricuspid atresia (63042009); Right atrioventricular valve atresia (253455004)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
HPO: HP:0011662
Monarch Initiative: MONDO:0011514
OMIM®: 605067
Orphanet: ORPHA1209

Definition

Failure to develop of the tricuspid valve and thus lack of the normal connection between the right atrium and the right ventricle. [from HPO]

Term Hierarchy

Conditions with this feature

Cat eye syndrome
MedGen UID:
120543
Concept ID:
C0265493
Disease or Syndrome
Cat eye syndrome (CES) is characterized clinically by the combination of coloboma of the iris and anal atresia with fistula, downslanting palpebral fissures, preauricular tags and/or pits, frequent occurrence of heart and renal malformations, and normal or near-normal mental development. A small supernumerary chromosome (smaller than chromosome 21) is present, frequently has 2 centromeres, is bisatellited, and represents an inv dup(22)(q11).
Holoprosencephaly-postaxial polydactyly syndrome
MedGen UID:
340382
Concept ID:
C1849649
Disease or Syndrome
Holoprosencephaly-postaxial polydactyly syndrome associates, in chromosomally normal neonates, holoprosencephaly, severe facial dysmorphism, postaxial polydactyly and other congenital abnormalities, suggestive of trisomy 13. Incidence is unknown. Dysmorphic features include hypotelorism, severe eye anomalies such as microphthalmia or anophthalmia, premaxillary region aplasia and cleft lip and palate. Congenital cardiac anomalies are common. The condition seems to be inherited as an autosomal recessive trait. Prognosis is poor.
Structural heart defects and renal anomalies syndrome
MedGen UID:
1387412
Concept ID:
C4479549
Disease or Syndrome
Feingold syndrome type 1
MedGen UID:
1637716
Concept ID:
C4551774
Disease or Syndrome
Feingold syndrome 1 (referred to as FS1 in this GeneReview) is characterized by digital anomalies (shortening of the 2nd and 5th middle phalanx of the hand, clinodactyly of the 5th finger, syndactyly of toes 2-3 and/or 4-5, thumb hypoplasia), microcephaly, facial dysmorphism (short palpebral fissures and micrognathia), gastrointestinal atresias (primarily esophageal and/or duodenal), and mild-to-moderate learning disability.
Cardiac valvular defect, developmental
MedGen UID:
1823949
Concept ID:
C5774175
Disease or Syndrome
Cardiac valvular dysplasia-1 (CVDP1) is characterized by congenital malformations of the pulmonic, tricuspid, and mitral valves. Structural cardiac defects, including atrial and ventricular septal defects, single left ventricle, and hypoplastic right ventricle have also been observed in affected individuals (Ta-Shma et al., 2017). Genetic Heterogeneity of Cardiac Valvular Dysplasia CVDP2 (620067) is caused by mutation in the ADAMTS19 gene (607513) on chromosome 5q23.

Professional guidelines

PubMed

Deeg KH
Ultraschall Med 2015 Apr;36(2):104-18; quiz 119-20. Epub 2014 Dec 4 doi: 10.1055/s-0034-1385493. PMID: 25474186
Rao PS
Indian J Pediatr 2009 Jan;76(1):57-70. Epub 2009 Apr 18 doi: 10.1007/s12098-009-0030-4. PMID: 19391004
Jaquiss RD, Imamura M
Semin Thorac Cardiovasc Surg 2007 Fall;19(3):258-63. doi: 10.1053/j.semtcvs.2007.07.004. PMID: 17983954

Recent clinical studies

Etiology

Dalldorf K, Parness I, Ezon D
Pediatr Cardiol 2022 Jun;43(5):1094-1103. Epub 2022 Feb 2 doi: 10.1007/s00246-022-02829-9. PMID: 35107630
Faber JW, Buijtendijk MFJ, Klarenberg H, Vink AS, Coolen BF, Moorman AFM, Christoffels VM, Clur SA, Jensen B
Pediatr Cardiol 2022 Apr;43(4):796-806. Epub 2022 Jan 6 doi: 10.1007/s00246-021-02789-6. PMID: 34988599
Backer CL, Mavroudis C
Methodist Debakey Cardiovasc J 2019 Apr-Jun;15(2):105-110. doi: 10.14797/mdcj-15-2-105. PMID: 31384373Free PMC Article
Zucker EJ, Koning JL, Lee EY
Radiol Clin North Am 2017 Jul;55(4):693-716. Epub 2017 Apr 6 doi: 10.1016/j.rcl.2017.02.009. PMID: 28601176
Keating P, Van der Merwe, Shipton
Cardiovasc J S Afr 2001 Aug-Sep;12(4):202-5. PMID: 11717697

Diagnosis

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
Sumal AS, Kyriacou H, Mostafa AMHAM
J Card Surg 2020 Jul;35(7):1609-1617. Epub 2020 Jun 2 doi: 10.1111/jocs.14673. PMID: 32484582
Deeg KH
Ultraschall Med 2015 Apr;36(2):104-18; quiz 119-20. Epub 2014 Dec 4 doi: 10.1055/s-0034-1385493. PMID: 25474186
Barata IA
Emerg Med Clin North Am 2013 Aug;31(3):677-704. doi: 10.1016/j.emc.2013.04.007. PMID: 23915599
Kreutzer GO, Vargas FJ, Schlichter AJ, Laura JP, Suarez JC, Coronel AR, Kreutzer EA
J Thorac Cardiovasc Surg 1982 Mar;83(3):427-36. PMID: 7062754

Therapy

AlZahrani A, Rathod R, Krimly A, Salam Y, AlMarzoog AT, Veldtman GR
Cardiol Clin 2020 Aug;38(3):379-401. doi: 10.1016/j.ccl.2020.05.002. PMID: 32622492
Sumal AS, Kyriacou H, Mostafa AMHAM
J Card Surg 2020 Jul;35(7):1609-1617. Epub 2020 Jun 2 doi: 10.1111/jocs.14673. PMID: 32484582
Backer CL, Mavroudis C
Methodist Debakey Cardiovasc J 2019 Apr-Jun;15(2):105-110. doi: 10.14797/mdcj-15-2-105. PMID: 31384373Free PMC Article
Kay WA, Moe T, Suter B, Tennancour A, Chan A, Krasuski RA, Zaidi AN
Prog Cardiovasc Dis 2018 Sep-Oct;61(3-4):365-376. Epub 2018 Sep 17 doi: 10.1016/j.pcad.2018.09.005. PMID: 30236751
Keating P, Van der Merwe, Shipton
Cardiovasc J S Afr 2001 Aug-Sep;12(4):202-5. PMID: 11717697

Prognosis

Faber JW, Buijtendijk MFJ, Klarenberg H, Vink AS, Coolen BF, Moorman AFM, Christoffels VM, Clur SA, Jensen B
Pediatr Cardiol 2022 Apr;43(4):796-806. Epub 2022 Jan 6 doi: 10.1007/s00246-021-02789-6. PMID: 34988599
AlZahrani A, Rathod R, Krimly A, Salam Y, AlMarzoog AT, Veldtman GR
Cardiol Clin 2020 Aug;38(3):379-401. doi: 10.1016/j.ccl.2020.05.002. PMID: 32622492
Sumal AS, Kyriacou H, Mostafa AMHAM
J Card Surg 2020 Jul;35(7):1609-1617. Epub 2020 Jun 2 doi: 10.1111/jocs.14673. PMID: 32484582
Anderson RH, Ho SY
Ann Thorac Surg 1998 Aug;66(2):644-8. doi: 10.1016/s0003-4975(98)00576-1. PMID: 9725445
Kreutzer GO, Vargas FJ, Schlichter AJ, Laura JP, Suarez JC, Coronel AR, Kreutzer EA
J Thorac Cardiovasc Surg 1982 Mar;83(3):427-36. PMID: 7062754

Clinical prediction guides

Suhl J, Conway KM, Rhoads A, Langlois PH, Feldkamp ML, Michalski AM, Oleson J, Sidhu A, Scholz TD, Kancherla V, Obrycki J, Mazumdar M, Romitti PA; National Birth Defects Prevention Study
Birth Defects Res 2023 Jan 1;115(1):79-87. Epub 2022 Nov 7 doi: 10.1002/bdr2.2110. PMID: 36341763Free PMC Article
Najashi KA, Najashi NA, Ahmed T, Abdelnaeem R, Alolit S, Zuaybir AA, Atiyah M, Trout AT, Alsaied T, Veldtman GR
Cardiol Young 2023 Oct;33(10):1834-1839. Epub 2022 Oct 19 doi: 10.1017/S1047951122002992. PMID: 36258282
Faber JW, Buijtendijk MFJ, Klarenberg H, Vink AS, Coolen BF, Moorman AFM, Christoffels VM, Clur SA, Jensen B
Pediatr Cardiol 2022 Apr;43(4):796-806. Epub 2022 Jan 6 doi: 10.1007/s00246-021-02789-6. PMID: 34988599
Sanchez-Quintana D, Climent V, Ho SY, Anderson RH
Heart 1999 Feb;81(2):182-91. doi: 10.1136/hrt.81.2.182. PMID: 9922357Free PMC Article
Van Praagh S, Vangi V, Sul JH, Metras D, Parness I, Castaneda AR, Van Praagh R
J Am Coll Cardiol 1991 Mar 15;17(4):932-43. doi: 10.1016/0735-1097(91)90876-b. PMID: 1999631

Recent systematic reviews

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
Kulkarni A, Patel N, Singh TP, Mossialos E, Mehra MR
J Heart Lung Transplant 2019 Jul;38(7):739-747. Epub 2019 Apr 4 doi: 10.1016/j.healun.2019.04.001. PMID: 31006521
Garcia Ropero A, Baskar S, Roos Hesselink JW, Girnius A, Zentner D, Swan L, Ladouceur M, Brown N, Veldtman GR
Circ Cardiovasc Qual Outcomes 2018 May;11(5):e004575. doi: 10.1161/CIRCOUTCOMES.117.004575. PMID: 29752389

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.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...