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hypoplastic right heart syndrome - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

hypoplastic right heart syndrome

MONDO:0020291

Also known as:right hypoplastic heart syndrome

Overview

Hypoplastic right-heart syndrome (HRHS) is a rare, cyanotic congenital heart malformation caused by underdevelopment of the right-sided heart structures (tricuspid valve, RV, pulmonary valve, and pulmonary artery) commonly associated with an atrial septal defect, ostium secundum type. Pulmonary blood flow is diminished and right-to-left shunting occurs at the atrial level, leading to dyspnea, fatigue, atrial arrhythmias, right-sided heart failure, hypoxemia, repeated miscarriages that were mostly due to hypoxemia and cyanosis. Two subtypes of HRHS have been characterized: pulmonary atresia-intact ventricular septum and right ventricular hypoplasia.

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 hypoplastic right heart syndrome.

14 publications have been identified in PubMed for hypoplastic right heart syndrome. Research spans Case Report / Case Series (64%), Review / Meta-Analysis (14%), and Epidemiology / Natural History (14%).

Research Type	Count	% of Total
Patient case studies	9	64%

Data assembled from 3 of 12 sources · Last updated Sep 19, 2026, 11:56 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:98723

GARD

Genetic and Rare Diseases Info Center

GARD:2922

Questions for Your Care Team

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hypoplastic right heart syndrome

Overview

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

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