Middle aortic syndrome
Synonyms: Abdominal coarctation · Coarctation of the abdominal aorta · MAC · Mid-aortic dysplastic syndrome · Mid-aortic syndrome · Midaortic syndrome · Middle aortic coarctation
- Type
- Morphological anomaly
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Childhood
- Status
- active
A rare vascular anomaly characterized by the segmental narrowing of the abdominal and/or distal descending thoracic aorta, with varying involvement of the visceral and renal arteries, that commonly presents in children and young adults with early onset and refractory hypertension, abdominal angina, and lower-limb claudication, that can lead to life-threatening complications associated with severe hypertension (i.e. myocardial infarction, heart failure, aortic rupture, renal insufficiency and intracranial hemorrhage). It may be due to various congenital or acquired causes, but it is most often secondary to an acquired inflammatory disease (i.e. Takayasu arteritis or giant cell arteritis).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Middle aortic syndrome | Abdominal coarctation · Coarctation of the abdominal aorta · MAC · Mid-aortic dysplastic syndrome · Mid-aortic syndrome · Midaortic syndrome · Middle aortic coarctation |
| fr | Coarctation atypique de l'aorte | Coarctation aortique atypique · Coarctation de l'aorte abdominale · Syndrome "mid-aortic" |
| de | Mid-Aortic-Syndrom | Aortenkoarktation, abdominale · Aortenkoarktation, mittlere · MAS |
| es | Síndrome aórtico medio | Coartación abdominal · Coartación aórtica media · Coartación de la aorta abdominal · Síndrome aorto-medial · Síndrome de aorta media · Síndrome displásico de aorta media |
| it | Sindrome dell'aorta media | Coartazione addominale · Coartazione dell'aorta addominale · Coartazione medioaortica · MAC · Sindrome medio-aortica · Sindrome medio-aortica displastica · Sindrome medioaortica |
| nl | Mid-aorta syndroom | Abdominale coarctatie · Coarctatie van abdominale aorta · Coarctatie van aorta abdominalis · MAC · Mid-aorta coarctatie · Mid-aorta dysplastisch syndroom |
| pt | Síndrome aórtico médio | Coartação abdominal · Coartação aórtica média · Coartação da aorta abdominal · MAC · Síndrome displásico aórtico médio · Síndrome médio-aórtico |
| pl | Zespół aorty brzusznej | Koarktacja aorty brzusznej · Zespół dysplastycznej aorty brzusznej · Zespół zwężenia aorty brzusznej · Zespół zwężenia aorty brzusznej · Zespół zwężenia aorty brzusznej |
| cs | Atypická koarktace aorty | Koarktace břišní aorty · Syndrom dysplázie střední aorty · Syndrom střední aorty · Syndrom břišní aorty |
| uk | Mid-aortic синдром (синдром звуження черевного відділу аорти) | Коарктація черевної порожнини · Коарктація черевної аорти · MAC · Диспластичний синдром середнього відділу аорти · Мідаортальний синдром · Синдром середнього відділу аорти · Коарктація середнього відділу аорти |
| zh | 非典型主动脉缩窄 | 腹主动脉缩窄 |
| tr | Aortun atipik koarktasyonu | Midaortik sendrom · Mid-aortik sendromu · Orta aortik sendromu · Mid-aortik displastik sendrom · Abdominal aortun koarktasyonu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q25.1 | NTBT | attributed | validated |
| ICD-11 | LA8B.21 | NTBT | index | validated |
| MONDO | 0015446 | E | validated | |
| UMLS | C3496579 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:98718Aortic malformation
rare developmental anomalies during embryogenesis; rare cardiac malformations; rare circulatory system diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Prevalence at birth | 1-9 / 1 000 000 | 0.17 / 100 000 | Europe | Value and class | Not yet validated | ORPHANET |
| Point prevalence | <1 / 1 000 000 | — | Europe | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/1456
- This record as JSON: /data/d/1456.json