Fixed subaortic stenosis

disease
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Summary

Fixed subaortic stenosis (MONDO:0017727) is a disease. A subtype of congenital anomaly of the great arteries — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

  • Prevalence: Unknown (Worldwide)
  • Phenotypes (HPO): 28

Clinical features

Signs & symptoms

Clinical features (HPO)

28 HPO clinical features (Orphanet curated; top 28 by frequency):

HPO IDTermFrequency
HP:0001627Abnormal heart morphologyFrequent (30-79%)
HP:0001629Ventricular septal defectFrequent (30-79%)
HP:0001635Congestive heart failureFrequent (30-79%)
HP:0001643Patent ductus arteriosusFrequent (30-79%)
HP:0001659Aortic regurgitationFrequent (30-79%)
HP:0001712Left ventricular hypertrophyFrequent (30-79%)
HP:0002094DyspneaFrequent (30-79%)
HP:0002875Exertional dyspneaFrequent (30-79%)
HP:0031664Systolic heart murmurFrequent (30-79%)
HP:0031668Diastolic heart murmurFrequent (30-79%)
HP:0032092Left ventricular outflow tract obstructionFrequent (30-79%)
HP:0001279SyncopeOccasional (5-29%)
HP:0001640CardiomegalyOccasional (5-29%)
HP:0001642Pulmonic stenosisOccasional (5-29%)
HP:0001647Bicuspid aortic valveOccasional (5-29%)
HP:0001653Mitral regurgitationOccasional (5-29%)
HP:0001671Abnormal cardiac septum morphologyOccasional (5-29%)
HP:0001680Coarctation of aortaOccasional (5-29%)
HP:0001681Angina pectorisOccasional (5-29%)
HP:0001962PalpitationsOccasional (5-29%)
HP:0004757Paroxysmal atrial fibrillationOccasional (5-29%)
HP:0004970Ascending tubular aorta aneurysmOccasional (5-29%)
HP:0006689Bacterial endocarditisOccasional (5-29%)
HP:0006695Atrioventricular canal defectOccasional (5-29%)
HP:0012764OrthopneaOccasional (5-29%)
HP:0030950Pulmonary venous hypertensionOccasional (5-29%)
HP:0030973Postexertional malaiseOccasional (5-29%)
HP:0100749Chest painOccasional (5-29%)

Identifiers

Disease identifiers

FieldValue
Canonical namefixed subaortic stenosis
Mondo IDMONDO:0017727
Orphanet3092
ICD-111471062257
UMLSC0265853
MedGen539538
GARD0016619
Is cancer (heuristic)no

Disease family

This is a subtype of congenital anomaly of the great arteries. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › cardiovascular disordercongenital anomaly of cardiovascular systemcongenital heart malformationcongenital anomaly of the great arteriesfixed subaortic stenosis

Related subtypes (14): aortic arch interruption, aortic arch defects, idiopathic pulmonary artery dilatation, scimitar syndrome, congenital pulmonary veins atresia or stenosis, congenital pulmonary valve stenosis, aorto-ventricular tunnel, aneurysm or dilatation of ascending aorta, premature closure of the arterial duct, absence of the pulmonary artery, congenital patent ductus arteriosus aneurysm, pulmonary artery hypoplasia, pulmonary branch stenosis, primary pulmonary vein stenosis

Subtypes (2): discrete fibromuscular subaortic stenosis, tunnel subaortic stenosis

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 0.

No linked Atlas pages yet — the cross-entity mesh grows as the corpus expands.