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fixed subaortic stenosis - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

fixed subaortic stenosis

MONDO:0017727

Overview

Fixed subaortic stenosis (FSS) is a rare heart malformation characterized by the obstruction by membranous or fibromuscular tissue of the left ventricular outflow tract (LVOT) below the aortic valve, that occurs as an isolated lesion or in association with additional cardiac malformations (e.g. ventricular septal defect, patent ductus arteriosus, coarctation of the aorta), that presents in childhood with signs of LVOT obstruction (e.g. dyspnea, chest pain, syncope, palpitations) and that can potentially lead to life-threatening complications (e.g. aortic regurgitation, infective endocarditis). It comprises three anatomical subforms: discrete fixed membranous subaortic stenosis (membranous tissue encircling the LVOT), discrete fibromuscular subaortic stenosis (fibromuscular tissue encircling the LVOT) and tunnel subaortic stenosis (fibromuscular diffuse tunnel-like narrowing of the LVOT), the two latter forms being generally more severe than the membranous form.

ResearchLimited published research (7 articles)

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 fixed subaortic stenosis.

7 publications have been identified in PubMed for fixed subaortic stenosis. Research spans Case Report / Case Series (71%), Review / Meta-Analysis (14%), and Epidemiology / Natural History (14%).

Recent Publications

Zambrano-Cevallos K (2026). [PMID: 42074916](https://pubmed.ncbi.nlm.nih.gov/42074916/). *J Clin Med*. [Case Report / Case Series]
Papatriantafyllou A (2026). [PMID: 41893448](https://pubmed.ncbi.nlm.nih.gov/41893448/). *Reports (MDPI)*. [Case Report / Case Series]
Arockiam AD (2025). [PMID: 41307634](https://pubmed.ncbi.nlm.nih.gov/41307634/). *Current cardiology reports*. [Review / Meta-Analysis]
Rocco R (2025). [PMID: 39822803](https://pubmed.ncbi.nlm.nih.gov/39822803/). *JACC. Case reports*. [Case Report / Case Series]
Cerbu M (2025). [PMID: 41409469](https://pubmed.ncbi.nlm.nih.gov/41409469/). *Frontiers in veterinary science*. [Case Report / Case Series]

Data assembled from 3 of 12 sources · Last updated Sep 18, 2026, 6:42 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:3092

GARD

Genetic and Rare Diseases Info Center

GARD:16619

Questions for Your Care Team

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fixed subaortic stenosis

Overview

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team