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isolated right ventricular hypoplasia (Irvh) - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

isolated right ventricular hypoplasia

MONDO:0010179

Also known as:Irvhhypoplasia of the right ventricleisolated hypoplasia of the right ventricle

Overview

Isolated right ventricular hypoplasia (IRVH) is a rare congenital heart malformation characterized by underdevelopment of the right ventricle associated with patent foramen ovale or interauricular communication and normally developed tricuspid and pulmonary valves. IRVH manifests with severe cyanosis, congestive heart failure, and in severe cases, death in early infancy.

GeneticsAutosomal recessive

Clinical Presentation

Features include: Hypoplasia of right ventricle.

Explore clinical features

Diagnosis

Biomarker and diagnostic research for isolated right ventricular hypoplasia has been reported in the published literature.

Prognosis

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.

No clinical trials have been registered for isolated right ventricular hypoplasia.

12 publications have been identified in PubMed for isolated right ventricular hypoplasia. Research spans Case Report / Case Series (75%), Other (8%), and Diagnostic / Biomarker (8%).

Research Type	Count	% of Total
Patient case studies	9	75%

Data assembled from 5 of 12 sources · Last updated Sep 18, 2026, 8:59 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:277200

Orphanet

European rare disease database

Orphanet:439

GARD

Genetic and Rare Diseases Info Center

GARD:4721

Questions for Your Care Team

→Should my partner and I meet with a genetic counselor to discuss carrier status and family planning?
→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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isolated right ventricular hypoplasia

Overview

Clinical Presentation

Explore clinical features

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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