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Tricuspid stenosis

MedGen UID:
21678
Concept ID:
C0040963
Disease or Syndrome
Synonyms: Stenoses, Tricuspid Valve; Stenosis, Tricuspid Valve; Tricuspid Valve Stenoses; Tricuspid Valve Stenosis; Valve Stenoses, Tricuspid; Valve Stenosis, Tricuspid
SNOMED CT: Tricuspid stenosis (49915006); TS - Tricuspid stenosis (49915006); Tricuspid valve stenosis (49915006)
 
HPO: HP:0010446
Monarch Initiative: MONDO:0005997

Definition

A narrowing of the orifice of the tricuspid valve of the heart. [from HPO]

Conditions with this feature

Congenital hypoplastic anemia
MedGen UID:
266045
Concept ID:
C1260899
Disease or Syndrome
Diamond-Blackfan anemia (DBA) is characterized by a profound normochromic and usually macrocytic anemia with normal leukocytes and platelets, congenital malformations in up to 50%, and growth deficiency in 30% of affected individuals. The hematologic complications occur in 90% of affected individuals during the first year of life. The phenotypic spectrum ranges from a mild form (e.g., mild anemia or no anemia with only subtle erythroid abnormalities, physical malformations without anemia) to a severe form of fetal anemia resulting in nonimmune hydrops fetalis. DBA is associated with an increased risk for acute myelogenous leukemia (AML), myelodysplastic syndrome (MDS), and solid tumors including osteogenic sarcoma.
Spondyloepiphyseal dysplasia with congenital joint dislocations
MedGen UID:
373381
Concept ID:
C1837657
Disease or Syndrome
CHST3-related skeletal dysplasia is characterized by short stature of prenatal onset, joint dislocations (knees, hips, radial heads), clubfeet, and limitation of range of motion that can involve all large joints. Kyphosis and occasionally scoliosis with slight shortening of the trunk develop in childhood. Minor heart valve dysplasia has been described in several persons. Intellect and vision are normal.
Diamond-Blackfan anemia 1
MedGen UID:
390966
Concept ID:
C2676137
Disease or Syndrome
Diamond-Blackfan anemia (DBA) is characterized by a profound normochromic and usually macrocytic anemia with normal leukocytes and platelets, congenital malformations in up to 50%, and growth deficiency in 30% of affected individuals. The hematologic complications occur in 90% of affected individuals during the first year of life. The phenotypic spectrum ranges from a mild form (e.g., mild anemia or no anemia with only subtle erythroid abnormalities, physical malformations without anemia) to a severe form of fetal anemia resulting in nonimmune hydrops fetalis. DBA is associated with an increased risk for acute myelogenous leukemia (AML), myelodysplastic syndrome (MDS), and solid tumors including osteogenic sarcoma.
Geleophysic dysplasia 1
MedGen UID:
479777
Concept ID:
C3278147
Disease or Syndrome
Geleophysic dysplasia, a progressive condition resembling a lysosomal storage disorder, is characterized by short stature, short hands and feet, progressive joint limitation and contractures, distinctive facial features, progressive cardiac valvular disease, and thickened skin. Intellect is normal. Major findings are likely to be present in the first year of life. Cardiac, respiratory, and lung involvement result in death before age five years in approximately 33% of individuals with ADAMTSL2-related geleophysic dysplasia.
Geleophysic dysplasia 2
MedGen UID:
481684
Concept ID:
C3280054
Disease or Syndrome
Geleophysic dysplasia, a progressive condition resembling a lysosomal storage disorder, is characterized by short stature, short hands and feet, progressive joint limitation and contractures, distinctive facial features, progressive cardiac valvular disease, and thickened skin. Intellect is normal. Major findings are likely to be present in the first year of life. Cardiac, respiratory, and lung involvement result in death before age five years in approximately 33% of individuals with ADAMTSL2-related geleophysic dysplasia.

Recent clinical studies

Etiology

Patel K, Sadeghi S, Aboulhosn J
World J Pediatr Congenit Heart Surg 2020 Jul;11(4):411-416. doi: 10.1177/2150135120910366. PMID: 32645780
Wang E, Fan X, Qi W, Song Y, Qi Z
Ann Thorac Surg 2019 Sep;108(3):e145-e147. Epub 2019 Mar 12 doi: 10.1016/j.athoracsur.2019.01.078. PMID: 30872099
Reddy YN, Connolly HM, Ammash NM
World J Pediatr Congenit Heart Surg 2015 Oct;6(4):667-9. doi: 10.1177/2150135115586270. PMID: 26467885
Lilly SM, Rome J, Anwaruddin S, Shreenivas S, Desai N, Silvestry FE, Herrmann HC, Fassa A, Himbert D, Brochet E, Labbé JP, Depoix JP, Hvass U, Vahanian A, Holoshitz N, Kenny D, Hijazi ZM
EuroIntervention 2013 Jul;9(3):407-9. doi: 10.4244/EIJV9I3A64. PMID: 23872655
Rosenberg Y, Myatt JP, Feldman M, Carpenter AJ, Bauch T, Restrepo CS, Panday M
Pacing Clin Electrophysiol 2010 May;33(5):e49-52. Epub 2010 Jan 12 doi: 10.1111/j.1540-8159.2009.02633.x. PMID: 20070545

Diagnosis

Patel K, Sadeghi S, Aboulhosn J
World J Pediatr Congenit Heart Surg 2020 Jul;11(4):411-416. doi: 10.1177/2150135120910366. PMID: 32645780
Randhawa VK, Vakamudi S, Phelan DM, Samaras CJ, McKenney JK, Hanna M, Perez AL
ESC Heart Fail 2020 Jun;7(3):1130-1135. Epub 2020 Mar 16 doi: 10.1002/ehf2.12668. PMID: 32175673Free PMC Article
Yang Z, Zhou L
Echocardiography 2019 Oct;36(10):1952-1955. Epub 2019 Aug 31 doi: 10.1111/echo.14466. PMID: 31471981
Shewale SD, Bhat P, Gupta AK, Manjunath CN
BMJ Case Rep 2016 Jul 19;2016 doi: 10.1136/bcr-2016-216373. PMID: 27436036Free PMC Article
Reddy YN, Connolly HM, Ammash NM
World J Pediatr Congenit Heart Surg 2015 Oct;6(4):667-9. doi: 10.1177/2150135115586270. PMID: 26467885

Therapy

Çetiner N, Baştuhan IY, Özyiğit T, Mutluer FO, Çeliker A
Turk J Pediatr 2020;62(4):681-684. doi: 10.24953/turkjped.2020.04.022. PMID: 32779425
Kulkarni SK, Moorthy N, Ramalingam R
Echocardiography 2019 Mar;36(3):598-601. Epub 2019 Jan 6 doi: 10.1111/echo.14254. PMID: 30614053
Munirathinam GK, Kumar B, Mishra AK
Ann Card Anaesth 2018 Apr-Jun;21(2):195-199. doi: 10.4103/aca.ACA_179_17. PMID: 29652285Free PMC Article
Essandoh M, Zuleta-Alarcon A, Weiss R, Sirak J, Dimitrova G, Wirtz C, Bhatt A, Castellon-Larios K
J Cardiothorac Vasc Anesth 2015 Apr;29(2):412-6. Epub 2014 Oct 25 doi: 10.1053/j.jvca.2014.07.013. PMID: 25440647
Rama-Merchan JC, Arribas-Jimenez A, Martin-Moreiras J, Garcia-Fernandez E, Cruz-Gonzalez I
Rev Port Cardiol 2014 Nov;33(11):739.e1-3. Epub 2014 Nov 4 doi: 10.1016/j.repc.2014.06.006. PMID: 25455950

Prognosis

Yang Z, Zhou L
Echocardiography 2019 Oct;36(10):1952-1955. Epub 2019 Aug 31 doi: 10.1111/echo.14466. PMID: 31471981
Wang E, Fan X, Qi W, Song Y, Qi Z
Ann Thorac Surg 2019 Sep;108(3):e145-e147. Epub 2019 Mar 12 doi: 10.1016/j.athoracsur.2019.01.078. PMID: 30872099
Nair A, Rajesh GN, Sajeev CG
Cardiol Young 2017 May;27(4):808-811. Epub 2017 Jan 12 doi: 10.1017/S1047951116002110. PMID: 28077182
Shewale SD, Bhat P, Gupta AK, Manjunath CN
BMJ Case Rep 2016 Jul 19;2016 doi: 10.1136/bcr-2016-216373. PMID: 27436036Free PMC Article
Divanović A, Witte D, Michelfelder E
Pediatr Cardiol 2012 Oct;33(7):1210-2. Epub 2012 Mar 8 doi: 10.1007/s00246-012-0253-4. PMID: 22398637

Clinical prediction guides

Yamamoto Y, Nomura K, Murayama F, Isobe S, Hoshino K
Pediatr Cardiol 2021 Dec;42(8):1854-1861. Epub 2021 Jul 13 doi: 10.1007/s00246-021-02679-x. PMID: 34255111
Ribeiro PA, al Zaibag M, Sawyer W
Eur Heart J 1989 Feb;10(2):120-6. doi: 10.1093/oxfordjournals.eurheartj.a059451. PMID: 2924781
Yousof AM, Shafei MZ, Endrys G, Khan N, Simo M, Cherian G
Am Heart J 1985 Jul;110(1 Pt 1):60-4. doi: 10.1016/0002-8703(85)90515-0. PMID: 4013991
Wooley CF, Fontana ME, Kilman JW, Ryan JM
Am J Med 1985 Mar;78(3):375-84. doi: 10.1016/0002-9343(85)90327-4. PMID: 3976700
Schlesinger P, Benchimol CB, Lopes AS, Barbosa J, Jasbik W, Benchimol AB
Clin Cardiol 1983 Apr;6(4):182-7. doi: 10.1002/clc.4960060406. PMID: 6839570

Recent systematic reviews

Yuan SM
Orphanet J Rare Dis 2020 Oct 28;15(1):305. doi: 10.1186/s13023-020-01561-y. PMID: 33115523Free PMC Article

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