Dextrocardia
- Type
- Morphological anomaly
- Group
- Disorder
- Age of onset
- Infancy, Neonatal
- Status
- active
A rare, congenital, non-syndromic, developmental defect during embryogenesis characterized by positioning of the heart in the right hemithorax, with the base and apex of the heart pointing caudally and to the right, due to abnormalities of embryologic origin that are intrinsic to the heart itself. Situs inversus or situs solitus may be associated, with extracardiac visceral transposition anomalies usually present in the former case and additional cardiac defects (e.g. septal defects, transposition of the great arteries, double-outlet right ventricle, anomalous pulmonary venous return, tetralogy of Fallot) frequently observed in both cases.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Dextrocardia | |
| fr | Dextrocardie | |
| de | Dextrokardie | |
| es | Dextrocardia | |
| it | Destrocardia | |
| nl | Dextrocardie | |
| pt | Dextrocardia | |
| pl | Dekstrokardia | |
| cs | Dextrokardie | |
| uk | Декстрокардія | |
| zh | 右位心 | |
| tr | Dekstrokardi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q24.0 | E | specific | validated |
| ICD-11 | LA80.1 | E | specific | validated |
| MONDO | 0015661 | E | validated | |
| MeSH | D003914 | E | validated | |
| UMLS | C0011813 | E | validated | |
| GARD | 1827 | E | validated | |
| MedDRA | 10012592 | 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:98716Heart position anomaly
rare developmental anomalies during embryogenesis; rare cardiac malformations
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | 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
26 annotations · validated 2017-07-12| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0001651 | Dextrocardia | |
| Very frequent (99-80%) | ||
| HP:0003115 | Abnormal EKG | |
| HP:0001627 | Abnormal heart morphology | |
| HP:0010872 | T-wave inversion | |
| Frequent (79-30%) | ||
| HP:0011603 | Congenital malformation of the great arteries | |
| HP:0001696 | Situs inversus totalis | |
| Occasional (29-5%) | ||
| HP:0002101 | Abnormal lung lobation | |
| HP:0012210 | Abnormal renal morphology | |
| HP:0012243 | Abnormal reproductive system morphology | |
| HP:0011620 | Abnormality of abdominal situs | |
| HP:0011615 | Abnormality of pulmonary situs | |
| HP:0004414 | Abnormality of the pulmonary artery | |
| HP:0001743 | Abnormality of the spleen | |
| HP:0000069 | Abnormality of the ureter | |
| HP:0002566 | Intestinal malrotation | |
| Very rare (<4-1%) | ||
| HP:0001760 | Abnormal foot morphology | |
| HP:0000772 | Abnormal rib morphology | |
| HP:0008771 | Aplasia/Hypoplasia of the ear | |
| HP:0001374 | Congenital hip dislocation | |
| HP:0001263 | Global developmental delay | |
| HP:0000238 | Hydrocephalus | |
| HP:0002245 | Meckel diverticulum | |
| HP:0003006 | Neuroblastoma | |
| HP:0002594 | Pancreatic hypoplasia | |
| HP:0000384 | Preauricular skin tag | |
| HP:0000465 | Webbed neck | |
Run the differential on the 4 obligate / very frequent terms
Annotation source: 25674744[PMID]_21861958[PMID]_17631088[PMID]_17515336[PMID]_15219529[PMID]_15219529[PMID]_13547381[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/1666
- This record as JSON: /data/d/1666.json