{"orpha":458718,"name":"Idiopathic spontaneous coronary artery dissection","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Idiopathic SCAD"],"definition":"A rare vascular disease characterized by idiopathic detachment of the layers of the walls of coronary arteries, creating a false lumen which limits the main coronary flow, leading to myocardial ischemia. Clinical manifestations include acute coronary syndromes, especially ST-segment elevation myocardial infarction (STEMI), syncope, cardiogenic shock, or sudden cardiac death. The condition typically affects young women.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-458718/","orphanet_url":"https://www.orpha.net/en/disease/detail/458718","names":{"fr":{"name":"Dissection spontanée idiopathique de l'artère coronaire","synonyms":["DSAC idiopathique"],"definition":"Maladie vasculaire rare caractérisée par un détachement idiopathique des couches de la paroi des artères coronaires, créant un faux chenal qui limite le flux coronaire principal, entraînant une ischémie myocardique. La maladie se manifeste par les syndromes coronariens aigus, en particulier l'infarctus du myocarde avec sus-décalage du segment ST (STEMI), la syncope, le choc cardiogénique ou la mort cardiaque subite. La maladie touche généralement les jeunes femmes."},"de":{"name":"Spontane Koronararteriendissektion","synonyms":["Idiopathische SCAD"],"definition":"Eine seltene Gefäßerkrankung, die durch eine idiopathische Ablösung der Wandschichten der Koronararterien gekennzeichnet ist, wodurch ein falsches Lumen entsteht, das den Hauptkoronarfluss einschränkt und zu einer Myokardischämie führt. Zu den klinischen Manifestationen gehören akute Koronarsyndrome, insbesondere der ST-Strecken-Hebungs-Myokardinfarkt (STEMI), Synkopen, kardiogener Schock oder plötzlicher Herztod. Von der Erkrankung sind in der Regel junge Frauen betroffen."},"es":{"name":"Disección coronaria espontánea idiopática","synonyms":["DCE idiopática","Disección espontánea de arterias coronarias idiopática"],"definition":"Es una enfermedad vascular poco frecuente caracterizada por un desprendimiento idiopático de las capas de las paredes de las arterias coronarias, dando lugar a una falsa luz que limita el flujo coronario principal, resultando en isquemia miocárdica. Las manifestaciones clínicas incluyen síndromes coronarios agudos, especialmente infarto de miocardio con elevación del segmento ST (STEMI), síncope, shock cardiogénico o muerte súbita cardíaca. Por lo general, el transtorno afecta a mujeres jóvenes."},"it":{"name":"Dissezione spontanea idiopatica dell'arteria coronaria","definition":"Si tratta di una malattia vascolare rara, caratterizzata dal distacco idiopatico degli strati delle pareti delle arterie coronarie, che creano un falso lume che limita il flusso coronarico principale, causando ischemia del miocardio. I segni clinici comprendono le sindromi coronariche acute, in particolare l'infarto del miocardio con sopra-slivellamento del segmento ST (STEMI), la sincope, lo shock cardiogeno e la morte cardiaca improvvisa. Di solito la malattia colpisce le donne giovani."},"nl":{"name":"Idiopathische spontane dissectie van kransslagaders","synonyms":["Idiopathische SCAD"],"definition":"Een zeldzame vasculaire aandoening, gekarakteriseerd door idiopathische loslating van lagen van kransslagaderwand, waardoor een vals lumen ontstaat dat de coronaire bloeddoorstroming beperkt, met ischemie van myocard tot gevolg. Klinische manifestaties zijn onder meer acute coronaire syndromen, vooral hartinfarct met elevatie van ST-segment (STEMI), syncope, cardiogene shock, of plotse hartdood. De aandoening treft doorgaans jonge vrouwen."},"pt":{"name":"Disseção espontânea idiopática da artéria coronária"},"pl":{"name":"Idiopatyczne spontaniczne rozwarstwienie tętnicy wieńcowej","synonyms":["Idiopatyczne SCAD"]},"cs":{"name":"Idiopatická spontánní disekce koronární artérie","synonyms":["Idiopatická SCAD"]},"uk":{"name":"Ідіопатичне спонтанне розшарування коронарних артерій","synonyms":["Ідіопатичний SCAD"],"definition":"A rare vascular disease characterized by idiopathic detachment of the layers of the walls of coronary arteries, creating a false lumen which limits the main coronary flow, leading to myocardial ischemia. Clinical manifestations include acute coronary syndromes, especially ST-segment elevation myocardial infarction (STEMI), syncope, cardiogenic shock, or sudden cardiac death. The condition typically affects young women."},"zh":{"name":"特发性自发性冠状动脉夹层","synonyms":["特发性SCAD"]},"tr":{"name":"İdiyopatik spontan koroner arter diseksiyonu","synonyms":["İdiopatik SCAD"]}},"xrefs":[{"source":"OMIM","code":"122455","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"I25.4","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"BA82","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0007385","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5679999","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10822","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97929,"name":"Rare cardiac disease"},"parents":[{"orpha":97929,"name":"Rare cardiac disease","classifications":[146]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}