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Atrial standstill

MedGen UID:
639047
Concept ID:
C0541782
Pathologic Function
Synonym: Sinus arrest
SNOMED CT: Sinus arrest (5609005); Atrial standstill (450919004); Atrial arrest (450919004)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Related genes: NPPA, GJA5
 
HPO: HP:0025478
Monarch Initiative: MONDO:0015281
Orphanet: ORPHA1344

Definition

Atrial standstill or silent atrium is a rare condition presenting with the absence of electrical and mechanical activity in the atria. It presents with the absence of P waves, bradycardia, and wide QRS complex in the electrocardiogram. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Conditions with this feature

Ebstein anomaly
MedGen UID:
4435
Concept ID:
C0013481
Congenital Abnormality
Ebstein anomaly is characterized by downward displacement of variable severity of the tricuspid valve into the right ventricle. The valve leaflets may be dysplastic, and a variable portion of the proximal part of the right ventricle is in continuity with the right atrium ('atrialized'), because of the abnormally positioned tricuspid valve. The severity of this defect includes a spectrum ranging from severe disturbance in fetal and neonatal life to virtually asymptomatic survival to adult life. Associated extracardiac anomalies in the setting of chromosomal or mendelian disorders occur in about 20% of patients with Ebstein anomaly. Nonsyndromic Ebstein anomaly can occur as a sporadic or a familial defect (summary by Digilio et al., 2011).
Catecholaminergic polymorphic ventricular tachycardia 1
MedGen UID:
351513
Concept ID:
C1631597
Disease or Syndrome
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is characterized by episodic syncope occurring during exercise or acute emotion. The underlying cause of these episodes is the onset of fast ventricular tachycardia (bidirectional or polymorphic). Spontaneous recovery may occur when these arrhythmias self-terminate. In other instances, ventricular tachycardia may degenerate into ventricular fibrillation and cause sudden death if cardiopulmonary resuscitation is not readily available. The mean onset of symptoms (usually a syncopal episode) is between age seven and 12 years; onset as late as the fourth decade of life has been reported. If untreated, CPVT is highly lethal, as approximately 30% of affected individuals experience at least one cardiac arrest and up to 80% have one or more syncopal spells. Sudden death may be the first manifestation of the disease.
Dilated cardiomyopathy 1E
MedGen UID:
331341
Concept ID:
C1832680
Disease or Syndrome
Any familial isolated dilated cardiomyopathy in which the cause of the disease is a mutation in the SCN5A gene.
Atrial standstill 2
MedGen UID:
816731
Concept ID:
C3810401
Disease or Syndrome
Atrial standstill (AS) is a rare condition characterized by the absence of electrical and mechanical activity in the atria. On surface ECG, AS is distinguished by bradycardia, junctional (usually narrow complex) escape rhythm, and absence of the P wave. Nearly 50% of patients with AS experience syncope. AS can be persistent or transient, and diffuse or partial (summary by Fazelifar et al., 2005).
Atrial standstill 1
MedGen UID:
1646392
Concept ID:
C4551959
Disease or Syndrome
Atrial standstill (AS) is a rare condition characterized by the absence of electrical and mechanical activity in the atria. On surface ECG, AS is distinguished by bradycardia, junctional (usually narrow complex) escape rhythm, and absence of the P wave. Nearly 50% of patients with AS experience syncope. AS can be persistent or transient, and diffuse or partial (summary by Fazelifar et al., 2005).

Professional guidelines

PubMed

Wang S, Peng D
Int Heart J 2019 Jan 25;60(1):12-18. Epub 2018 Dec 5 doi: 10.1536/ihj.17-604. PMID: 30518714
Hofman-Bang J, Behr ER, Hedley P, Tfelt-Hansen J, Kanters JK, Haunsøe S, McKenna WJ, Christiansen M
Clin Genet 2006 Jun;69(6):504-11. doi: 10.1111/j.1399-0004.2006.00621.x. PMID: 16712702
Waldo AL, Vitikainen KJ, Kaiser GA, Bowman FO Jr, Malm JR
Circulation 1972 Oct;46(4):690-7. doi: 10.1161/01.cir.46.4.690. PMID: 5072770

Recent clinical studies

Etiology

Howard TS, Chiang DY, Ceresnak SR, Ladouceur VB, Whitehill RD, Czosek RJ, Knilans TK, Ahnfeldt AM, Borresen ML, Jaeggi E, Udupa S, Gow R, Moore JP, Galloti RG, Mah DY, Kim JJ, Valdes SO, Milewicz DM, Miyake CY
JACC Clin Electrophysiol 2023 Jan;9(1):57-69. Epub 2022 Oct 26 doi: 10.1016/j.jacep.2022.08.022. PMID: 36435694
Chen J, Ma Y, Li H, Lin Z, Yang Z, Zhang Q, Wang F, Lin Y, Ye Z, Lin Y
Orphanet J Rare Dis 2022 May 7;17(1):183. doi: 10.1186/s13023-022-02348-z. PMID: 35526016Free PMC Article
Wang S, Peng D
Int Heart J 2019 Jan 25;60(1):12-18. Epub 2018 Dec 5 doi: 10.1536/ihj.17-604. PMID: 30518714
Boriani G, Biagini E, Ziacchi M, Malavasi VL, Vitolo M, Talarico M, Mauro E, Gorlato G, Lattanzi G
Nucleus 2018;9(1):442-459. doi: 10.1080/19491034.2018.1506680. PMID: 30130999Free PMC Article
Zaklyazminskaya E, Dzemeshkevich S
Biochim Biophys Acta 2016 Jul;1863(7 Pt B):1799-805. Epub 2016 Feb 23 doi: 10.1016/j.bbamcr.2016.02.014. PMID: 26916278

Diagnosis

Howard TS, Chiang DY, Ceresnak SR, Ladouceur VB, Whitehill RD, Czosek RJ, Knilans TK, Ahnfeldt AM, Borresen ML, Jaeggi E, Udupa S, Gow R, Moore JP, Galloti RG, Mah DY, Kim JJ, Valdes SO, Milewicz DM, Miyake CY
JACC Clin Electrophysiol 2023 Jan;9(1):57-69. Epub 2022 Oct 26 doi: 10.1016/j.jacep.2022.08.022. PMID: 36435694
Ferreira J, Martins J, Gonçalves L
Cardiovasc J Afr 2021 Mar-Apr;32(2):108-110. doi: 10.5830/CVJA-2020-026. PMID: 34143176Free PMC Article
Wang S, Peng D
Int Heart J 2019 Jan 25;60(1):12-18. Epub 2018 Dec 5 doi: 10.1536/ihj.17-604. PMID: 30518714
John RM, Kumar S
Circulation 2016 May 10;133(19):1892-900. doi: 10.1161/CIRCULATIONAHA.116.018011. PMID: 27166347
Ruff P, Leier CV, Schaal SF
Am Heart J 1979 Oct;98(4):413-20. doi: 10.1016/0002-8703(79)90245-x. PMID: 484429

Therapy

Wang S, Peng D
Int Heart J 2019 Jan 25;60(1):12-18. Epub 2018 Dec 5 doi: 10.1536/ihj.17-604. PMID: 30518714
Ideguchi T, Tsuruda T, Sato Y, Kitamura K
Circ J 2016 Jul 25;80(8):1781-6. Epub 2016 Jun 13 doi: 10.1253/circj.CJ-16-0283. PMID: 27301330
John RM, Kumar S
Circulation 2016 May 10;133(19):1892-900. doi: 10.1161/CIRCULATIONAHA.116.018011. PMID: 27166347
Álvarez-García J, Vives-Borrás M, Gomis P, Ordoñez-Llanos J, Ferrero-Gregori A, Serra-Peñaranda A, Cinca J
Circulation 2016 Jun 7;133(23):2235-42. Epub 2016 May 5 doi: 10.1161/CIRCULATIONAHA.116.021700. PMID: 27151531
Kambara H, Phillips JH
Angiology 1976 Jul-Aug;27(7):455-62. doi: 10.1177/000331977602700706. PMID: 1078321

Prognosis

Chen J, Ma Y, Li H, Lin Z, Yang Z, Zhang Q, Wang F, Lin Y, Ye Z, Lin Y
Orphanet J Rare Dis 2022 May 7;17(1):183. doi: 10.1186/s13023-022-02348-z. PMID: 35526016Free PMC Article
Álvarez-García J, Vives-Borrás M, Gomis P, Ordoñez-Llanos J, Ferrero-Gregori A, Serra-Peñaranda A, Cinca J
Circulation 2016 Jun 7;133(23):2235-42. Epub 2016 May 5 doi: 10.1161/CIRCULATIONAHA.116.021700. PMID: 27151531
Disertori M, Quintarelli S, Grasso M, Pilotto A, Narula N, Favalli V, Canclini C, Diegoli M, Mazzola S, Marini M, Del Greco M, Bonmassari R, Masè M, Ravelli F, Specchia C, Arbustini E
Circ Cardiovasc Genet 2013 Feb;6(1):27-36. Epub 2012 Dec 29 doi: 10.1161/CIRCGENETICS.112.963520. PMID: 23275345
Türkoğlu C, Aliyev F, Celiker C, Fıratlı I
Turk Kardiyol Dern Ars 2011 Jan;39(1):55-8. PMID: 21358233
Nakazato Y, Nakata Y, Hisaoka T, Sumiyoshi M, Ogura S, Yamaguchi H
Pacing Clin Electrophysiol 1995 Jun;18(6):1244-54. doi: 10.1111/j.1540-8159.1995.tb06964.x. PMID: 7659578

Clinical prediction guides

Chen J, Ma Y, Li H, Lin Z, Yang Z, Zhang Q, Wang F, Lin Y, Ye Z, Lin Y
Orphanet J Rare Dis 2022 May 7;17(1):183. doi: 10.1186/s13023-022-02348-z. PMID: 35526016Free PMC Article
Ishikawa T, Mishima H, Barc J, Takahashi MP, Hirono K, Terada S, Kowase S, Sato T, Mukai Y, Yui Y, Ohkubo K, Kimoto H, Watanabe H, Hata Y, Aiba T, Ohno S, Chishaki A, Shimizu W, Horie M, Ichida F, Nogami A, Yoshiura KI, Schott JJ, Makita N
Circ Arrhythm Electrophysiol 2020 Oct;13(10):e008712. Epub 2020 Jul 29 doi: 10.1161/CIRCEP.120.008712. PMID: 32755394
Álvarez-García J, Vives-Borrás M, Gomis P, Ordoñez-Llanos J, Ferrero-Gregori A, Serra-Peñaranda A, Cinca J
Circulation 2016 Jun 7;133(23):2235-42. Epub 2016 May 5 doi: 10.1161/CIRCULATIONAHA.116.021700. PMID: 27151531
Tan HL
J Cardiovasc Electrophysiol 2006 May;17 Suppl 1:S151-S157. doi: 10.1111/j.1540-8167.2006.00398.x. PMID: 16686672
Kambara H, Phillips JH
Angiology 1976 Jul-Aug;27(7):455-62. doi: 10.1177/000331977602700706. PMID: 1078321

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