ORPHA:99104

Atrial septal defect, coronary sinus type

Synonyms: ASD, coronary sinus type · Unroofed coronary sinus

This disease is described under Interatrial communication

Names in Orphadata languages

12 of 12 languages

Lang	Name	Synonyms
en	Atrial septal defect, coronary sinus type	ASD, coronary sinus type · Unroofed coronary sinus
fr	Communication interauriculaire type sinus coronaire	ASD type sinus coronaire · CIA type sinus coronaire · Communication interauriculaire avec fenestration du toit du sinus coronaire
de	Atriumseptumdefekt, Koronarsinus Typ	ASD vom Koronarsinus-Typ · Koronarsinus-Defekt · Vorhofseptumdefekt vom Koronarsinus-Typ
es	Comunicación interauricular, tipo seno coronario	CIA, tipo seno coronario · Seno coronario destechado
it	Fistola coronarica, tipo DIA
nl	Atriaal septaal defect, coronaire sinus-type	ASD, coronaire sinus-type · Niet afgedekte coronaire sinus
pt	Defeito do septo auricular, tipo seio coronário	Seio coronário, tipo ASD
pl	Ubytek przegrody międzyprzedsionkowej, typ zatoki wieńcowej	ASD, typ zatoki wieńcowej · Nieukorzeniona zatoka wieńcowa
cs	Defekt síňového septa, typ sinus coronarius	ASD, typ sinus coronarius · Nezastřešený sinus coronarius
uk	ВВС. Дефект міжпередсердної перетинки по типу дефекта коронарного синуса (непокритий коронарний синус)	ASD, коронарний синусовий тип · Незароговілий коронарний синус
zh	房间隔缺损冠状静脉窦型	冠状窦型房间隔缺损
tr	Atriyal septal defekt, koroner sinüs tipi	Çatısız koroner sinüs · ASD, koroner sinüs tipi

qualifier = Orphanet mapping relation

Source	Code	Qualifier	ICD relation	Validation
ICD-10	Q21.1	NTBT	inclusion	validated
ICD-11	LA8E.3	E	specific	validated
MONDO	0020435	E		validated
UMLS	C0344733	E		validated
GARD	10697	E		validated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

ORPHA:1478Interatrial communication
rare developmental anomalies during embryogenesis; rare cardiac malformations; rare genetic diseases

No children.

0 direct

Orphanet records no gene directly on this code.

0 records

No prevalence record in this release.

26 annotations · validated 2020-03-10

HPO	Term (as annotated by Orphanet)	Diagnostic
Very frequent (99-80%)
HP:0012382	Left-to-right shunt
HP:0031297	Unroofed coronary sinus
Frequent (79-30%)
HP:0031687	Abnormally loud pulmonic component of the second heart sound
HP:0031634	Anomalous origin of the left common carotid artery from the main pulmonary artery
HP:0010772	Anomalous pulmonary venous return
HP:0003546	Exercise intolerance
HP:0002875	Exertional dyspnea
HP:0012378	Fatigue
HP:0001962	Palpitations
HP:0031664	Systolic heart murmur
Occasional (29-5%)
HP:0011675	Arrhythmia
HP:0011710	Bundle branch block
HP:0002094	Dyspnea
HP:0031972	Presyncope
HP:0002092	Pulmonary arterial hypertension
HP:0030718	Right atrial enlargement
HP:0005133	Right ventricular dilatation
HP:0005115	Supraventricular arrhythmia
HP:0002326	Transient ischemic attack
Very rare (<4-1%)
HP:0000961	Cyanosis
HP:0005317	Increased pulmonary vascular resistance
HP:0002090	Pneumonia
HP:0002718	Recurrent bacterial infections
HP:0001708	Right ventricular failure
HP:0001297	Stroke
HP:0001279	Syncope

Annotation source: 9374080[PMID]_22122378[PMID]_27161562[PMID]_30571061[PMID]