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univentricular heart - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

univentricular heart

MONDO:0015451

Also known as:Double inlet left ventricle

Find Clinical TrialsTrials

Overview

Univentricular heart (UVH) is a severe congenital cardiac malformation characterized by both atria related entirely or almost entirely to one functionally single ventricular chamber. The clinical manifestations include congestive heart failure, failure to thrive, cyanosis, hypoxemia and neurodevelopmental disabilities.

Clinical Trials9 registered · 5 recruiting

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include very common findings: Pulmonary venous hypertension and Valvular pulmonary stenosis; and common findings: Failure to thrive, Congestive heart failure, Respiratory distress, and Tachypnea and others. 14 total HPO annotations.

Organ System	Phenotype Count	Example Features
Heart and blood vessels	7	Pulmonary venous hypertension, Valvular pulmonary stenosis, Congestive heart failure

Diagnosis

Biomarker and diagnostic research for univentricular heart has been reported in the published literature.

Prognosis

Phenotype severity distribution: 2 very common features, 6 common features.

Estimated prevalence: Unknown (Unknown prevalence).

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.

9 clinical trials registered, 5 recruiting. Interventions under study include other interventions, drug therapy, medical devices, and procedural interventions. Pipeline includes 1 PHASE2, 1 PHASE1, 1 EARLY_PHASE1. Research is sponsored by a mix of industry and academic institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT05563376](https://clinicaltrials.gov/study/NCT05563376)

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 6:44 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:1464

GARD

Genetic and Rare Diseases Info Center

GARD:18725

Questions for Your Care Team

→Are any of the 9 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?

← Back to All Diseases

Research Type	Count	% of Total
Disease patterns and progression	27	29%
Clinical study results	26	28%
Research summaries	17	18%
Patient case studies	9	10%
Laboratory research	7	7%
Testing and diagnosis research	4	4%
Other research	2	2%
New treatment approaches	2	2%

Kisho

univentricular heart

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications

univentricular heart

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications