Idiopathic neonatal atrial flutter

General Information (adopted from Orphanet):

Synonyms, Signs:
Number of Symptoms 8
OrphanetNr: 45452
OMIM Id:
ICD-10: I48
UMLs:
MeSH:
MedDRA:
Snomed:

Prevalence, inheritance and age of onset:

Prevalence: No data available.
Inheritance:
Age of onset: Neonatal
Infancy
9060798 [IBIS]

Disease classification (adopted from Orphanet):

Parent Diseases: Non-genetic cardiac rhythm disease
 -Rare cardiac disease

Comment:

Idiopathic neonatal atrial flutter (AFL) is characterized by sustained tachycardia in newborns and infants. The majority of patients present with symptoms within the first 48 h of life. (PMID:9060798)

Symptom Information: Sort by abundance 

1
(HPO:0001629) Ventricular septal defect Rare [IBIS] 9060798 IBIS 316 / 7739
2
(HPO:0001643) Patent ductus arteriosus Rare [IBIS] 9060798 IBIS 228 / 7739
3
(HPO:0005150) Abnormal atrioventricular conduction Frequent [IBIS] 64% (n=25) 9060798 IBIS 16 / 7739
4
(HPO:0004749) Atrial flutter Very frequent [IBIS] 100% (n=25) 9060798 IBIS 20 / 7739
5
(HPO:0001649) Tachycardia Frequent [IBIS] 80% (n=25) 9060798 IBIS 53 / 7739
6
(HPO:0001635) Congestive heart failure Frequent [IBIS] 36% (n=25) 9060798 IBIS 232 / 7739
7
(HPO:0001645) Sudden cardiac death Occasional [IBIS] 8% (n=25) 9060798 IBIS 84 / 7739
8
(HPO:0001789) Hydrops fetalis Occasional [IBIS] 20% (n=25) 9060798 IBIS 63 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference
NUP155 rs587777339 pathogenic RCV000114997.3

Additional Information: