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tricuspid atresia - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

tricuspid atresia

MONDO:0011514

Also known as:congenital atresia of tricuspid valvetricuspid atresia (disease)tricuspid valve atresia

Find Clinical TrialsTrials

Overview

Tricuspid atresia is (TA) a rare congenital heart malformation characterized by the congenital agenesis of tricuspid valve leading to severe hypoplasia of right ventricle (functionally univentricular). TA is associated with normally related or transposed great vessels (TGV), an obligatory interatrial connection that is crucial for survival (patent foramen ovale or atrial septal defect, osteum secondum type), ventricular septal defect (in 90% cases), pulmonary outflow obstruction - pulmonary atresia, stenosis or hypoplasia (usually in TA with normally related vessels but also in TGV), aortic coarctation and/or aortic arch interruption (usually in TA with TGV).

Clinical Trials2 registered · 2 recruiting

Diagnosis

Biomarker and diagnostic research for tricuspid atresia has been reported in the published literature.

Prognosis

Estimated prevalence: 1-9 in 100,000 (Uncommon).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

2 clinical trials registered, 2 recruiting. Interventions under study include drug therapy and medical devices. Pipeline includes 1 PHASE2. Research is primarily sponsored by academic and government institutions.

117 publications have been identified in PubMed for tricuspid atresia. Research spans Case Report / Case Series (39%), Epidemiology / Natural History (23%), and Review / Meta-Analysis (12%).

Research Type	Count	% of Total
Patient case studies	45

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 4:18 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:605067

Orphanet

European rare disease database

Orphanet:1209

GARD

Genetic and Rare Diseases Info Center

GARD:5274

Questions for Your Care Team

→Are any of the 2 active clinical trials a good fit for my situation?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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tricuspid atresia

Overview

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

OMIM

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

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