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Hypoplastic tricuspid valve

MedGen UID:
472995
•Concept ID:
C0265837
•
Congenital Abnormality
Synonym: Tricuspid valve hypoplasia
SNOMED CT: Congenital hypoplasia of tricuspid valve (43176009); Congenital hypoplasia of right atrioventricular valve (43176009); Congenital small right atrioventricular valve (43176009)
 
HPO: HP:0011573

Definition

Congenital defect characterized by underdevelopment of the tricuspid valve. [from HPO]

Term Hierarchy

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

Conditions with this feature

Pancreatic hypoplasia-diabetes-congenital heart disease syndrome
MedGen UID:
860891
•Concept ID:
C4012454
•
Congenital Abnormality
A rare, syndromic diabetes mellitus characterized by partial pancreatic agenesis, diabetes mellitus, and heart anomalies (including transposition of the great vessels, ventricular or atrial septal defects, pulmonary stenosis, or patent ductus arteriosis).
See: Condition Record
Pancreatic hypoplasia-diabetes-congenital heart disease syndrome

Professional guidelines

PubMed

Prenatal diagnosis and pregnancy outcome of major structural anomalies detectable in the first trimester: A population-based cohort study in the Netherlands.
Bardi F, Bergman JEH, Siemensma-Mühlenberg N, Elvan-Taşpınar A, de Walle HEK, Bakker MK
Paediatr Perinat Epidemiol 2022 Nov;36(6):804-814. Epub 2022 Jul 12 doi: 10.1111/ppe.12914. PMID: 35821640Free PMC Article
Prenatal diagnosis of functionally univentricular heart, associations and perinatal outcomes.
Wolter A, Nosbüsch S, Kawecki A, Degenhardt J, Enzensberger C, Graupner O, Vorisek C, Akintürk H, Yerebakan C, Khalil M, Schranz D, Ritgen J, Stressig R, Axt-Fliedner R
Prenat Diagn 2016 Jun;36(6):545-54. Epub 2016 May 10 doi: 10.1002/pd.4821. PMID: 27061183
Echocardiographic differential diagnosis of the cyanotic newborn.
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

Recent clinical studies

Etiology

Pattern and clinical profile of children with complex cardiac anomaly at University of Nigeria Teaching Hospital, Ituku-Ozalla, Enugu State, Nigeria.
Chinawa JM, Obu HA, Eke CB, Eze JC
Niger J Clin Pract 2013 Oct-Dec;16(4):462-7. doi: 10.4103/1119-3077.116890. PMID: 23974740
Arterial switch with internal pulmonary artery banding. A new palliation for TGA and VSD in complex cases.
Conte S, Jensen T, Ramsøe Jacobsen J, Lauridsen P, Pettersson G
J Cardiovasc Surg (Torino) 1999 Apr;40(2):313-6. PMID: 10350125

Diagnosis

Balloon valvuloplasty for annular- and leaflet-level tricuspid stenosis: Report of cases and literature review.
Ephrem G, Gorocica-Romero R, Day J, Flores-Umanzor E, Abrahamyan L, Horlick E
J Invasive Cardiol 2024 Jan;36(1) doi: 10.25270/jic/23.00205. PMID: 38224293
A case of staged Norwood procedure for a unique form of truncus arteriosus, interrupted aortic arch with hypoplastic tricuspid valve and right ventricle and anomalous left coronary artery orifice: first reported case.
Fujimoto Y, Tachi M, Suehiro S, Ito M, Oda T
Gen Thorac Cardiovasc Surg 2017 Apr;65(4):209-212. Epub 2015 Dec 19 doi: 10.1007/s11748-015-0608-x. PMID: 26688560
Coexistent cardiac tumours and malformations of the heart.
Russell GA, Dhasmana JP, Berry PJ, Gilbert-Barness EF
Int J Cardiol 1989 Jan;22(1):89-98. doi: 10.1016/0167-5273(89)90140-x. PMID: 2925289
Pulmonary atresia with intact ventricular septum, Ebstein's anomaly of hypoplastic tricuspid valve, and double-chamber right ventricle: two-dimensional echocardiographic-anatomic correlation.
Huhta JC, Edwards WD, Tajik AJ, Mair DD, Puga FJ, Ritter DG
Mayo Clin Proc 1982 Aug;57(8):515-9. PMID: 7098532

Prognosis

Coexistent cardiac tumours and malformations of the heart.
Russell GA, Dhasmana JP, Berry PJ, Gilbert-Barness EF
Int J Cardiol 1989 Jan;22(1):89-98. doi: 10.1016/0167-5273(89)90140-x. PMID: 2925289

Clinical prediction guides

Arterial switch with internal pulmonary artery banding. A new palliation for TGA and VSD in complex cases.
Conte S, Jensen T, Ramsøe Jacobsen J, Lauridsen P, Pettersson G
J Cardiovasc Surg (Torino) 1999 Apr;40(2):313-6. PMID: 10350125

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