Details of Disease | DrugMAP

General Information of Disease (ID: DISFHGZA)

Disease Name	Atrial standstill
Synonyms	atrial cardiomyopathy with heart block
Definition	Atrial standstill is a rare cardiac rhythm disease with a few familial and sporadic cases described to date that is characterized by a transient or permanent absence of electrical and mechanical atrial activity. Electrocardiographic findings include bradycardia, ectopic supraventricular rhythms, lack of atrial excitability and absent P waves.
Disease Hierarchy	DISL4MMU: Familial restrictive cardiomyopathy DISFHGZA: Atrial standstill
Disease Identifiers	MONDO ID MONDO_0015281 UMLS CUI C0541782 MedGen ID 639047 HPO ID HP:0025478 Orphanet ID 1344 SNOMED CT ID 450919004

Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 2 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
SCN5A	TTZOVE0	Limited	GermlineCausalMutation	[1]
SCN5A	TTZOVE0	Supportive	Autosomal dominant	[1]
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This Disease Is Related to 2 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
NPPA	OTMQNTNX	Supportive	Autosomal dominant	[2]
SCN5A	OTGYZWR6	Supportive	Autosomal dominant	[1]
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References

1	A cardiac sodium channel mutation cosegregates with a rare connexin40 genotype in familial atrial standstill. Circ Res. 2003 Jan 10;92(1):14-22. doi: 10.1161/01.res.0000050585.07097.d7.
2	Autosomal recessive atrial dilated cardiomyopathy with standstill evolution associated with mutation of Natriuretic Peptide Precursor A. Circ Cardiovasc Genet. 2013 Feb;6(1):27-36. doi: 10.1161/CIRCGENETICS.112.963520. Epub 2012 Dec 29.