loading...| Preferred Name |
Endocardial fibroelastosis |
| ID |
http://www.orpha.net/ORDO/Orphanet_2022 |
| alternative_term |
Endomyocardial fibroelastosis |
| definition |
Endomyocardial fibroelastosis is a cause of unexplained childhood cardiac insufficiency. It results from diffuse thickening of the endocardium leading to dilated myocardiopathy in the majority of cases and restrictive myocardiopathy in rare cases. It may occur as a primary disorder or may be secondary to another cardiac malformation, notably aortic stenosis or atresia. |
| definition_citation |
Orphanet |
| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=2022 |
| has_age_of_onset |
Neonatal |
| has_inheritance |
Unknown |
| hasDbXref |
MedDRA:10014663 ICD-10:I42.4 ICD-11:BC43.3 UMLS:C0014117 OMIM:226000 |
| label |
Endocardial fibroelastosis |
| notation |
ORPHA:2022 |
| part_of |
http://www.orpha.net/ORDO/Orphanet_506225 |
| prefixIRI |
ORDO:Orphanet_2022 |
| prefLabel |
Endocardial fibroelastosis |
| present_in |
Worldwide AND has_point_prevalence_range : Unknown |
| treeView |
http://www.orpha.net/ORDO/Orphanet_506225 |
| subClassOf |