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Atrial standstill 1(ATRST1)

MedGen UID:
1646392
•Concept ID:
C4551959
•
Disease or Syndrome
Synonyms: ATRIAL CARDIOMYOPATHY WITH HEART BLOCK; Atrial standstill, digenic (GJA5/SCN5A); ATRST1; CARDIOMYOPATHY, FAMILIAL, WITH CONDUCTION DISTURBANCE
 
Gene (location): GJA5 (1q21.2)
 
Monarch Initiative: MONDO:0007171
OMIM®: 108770

Definition

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). [from OMIM]

Clinical features

From HPO
Endocardial fibroelastosis
MedGen UID:
4041
•Concept ID:
C0014117
•
Disease or Syndrome
Diffuse thickening of the ventricular endocardium and by associated myocardial dysfunction
See: Feature record | Search on this feature
Premature atrial contractions
MedGen UID:
19455
•Concept ID:
C0033036
•
Disease or Syndrome
A type of cardiac arrhythmia with premature atrial contractions or beats caused by signals originating from ectopic atrial sites.
See: Feature record | Search on this feature
First degree atrioventricular block
MedGen UID:
43215
•Concept ID:
C0085614
•
Disease or Syndrome
Delay of conduction through the atrioventricular node, which is manifested as prolongation of the PR interval in the electrocardiogram (EKG). All atrial impulses reach the ventricles.
See: Feature record | Search on this feature
Ventricular escape rhythm
MedGen UID:
65423
•Concept ID:
C0232216
•
Pathologic Function
A ventricular escape rhythm occurs whenever higher-lever pacemakers in AV junction or sinus node fail to control ventricular activation. Escape rate is usually 20-40 bpm, often associated with broad QRS complexes (at least 120 ms).
See: Feature record | Search on this feature
Paroxysmal atrial fibrillation
MedGen UID:
115990
•Concept ID:
C0235480
•
Disease or Syndrome
Episodes of atrial fibrillation that typically last for several hours up to one day and terminate spontaneously.
See: Feature record | Search on this feature
Atrial standstill
MedGen UID:
639047
•Concept ID:
C0541782
•
Pathologic Function
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.
See: Feature record | Search on this feature
Atrial cardiomyopathy
MedGen UID:
867504
•Concept ID:
C4021885
•
Disease or Syndrome
Any complex of structural, architectural, contractile or electrophysiological changes affecting the atria with the potential to produce clinically relevant manifestations.
See: Feature record | Search on this feature

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Professional guidelines

PubMed

Cardiac Involvement in Emery-Dreifuss Muscular Dystrophy and Related Management Strategies.
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

Recent clinical studies

Etiology

Atrial Standstill in the Pediatric Population: A Multi-Institution Collaboration.
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
Cardiac Involvement in Emery-Dreifuss Muscular Dystrophy and Related Management Strategies.
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
Cardiolaminopathies from bench to bedside: challenges in clinical decision-making with focus on arrhythmia-related outcomes.
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
Coexisting Hyponatremia and Decline in Diastolic Blood Pressure Predispose to Atrial Standstill in Hyperkalemic Patients.
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
The role of mutations in the SCN5A gene in cardiomyopathies.
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

Atrial Standstill in the Pediatric Population: A Multi-Institution Collaboration.
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
Cardiac Involvement in Emery-Dreifuss Muscular Dystrophy and Related Management Strategies.
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
Cardiolaminopathies from bench to bedside: challenges in clinical decision-making with focus on arrhythmia-related outcomes.
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
Electrophysiological Effects of Selective Atrial Coronary Artery Occlusion in Humans.
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
A cardiac sodium channel mutation cosegregates with a rare connexin40 genotype in familial atrial standstill.
Groenewegen WA, Firouzi M, Bezzina CR, Vliex S, van Langen IM, Sandkuijl L, Smits JP, Hulsbeek M, Rook MB, Jongsma HJ, Wilde AA
Circ Res 2003 Jan 10;92(1):14-22. doi: 10.1161/01.res.0000050585.07097.d7. PMID: 12522116

Therapy

Cardiac and renal AL amyloidosis controlled by autologous stem cell transplantation for 17 years accompanying late onset atrial fibrillation and complete atrioventricular block.
Iijima T, Sawa N, Oba Y, Ikuma D, Mizuno H, Yamanouchi M, Suwabe T, Wake A, Kono K, Ubara Y, Ohashi K
CEN Case Rep 2023 Nov;12(4):362-367. Epub 2023 Feb 16 doi: 10.1007/s13730-023-00777-8. PMID: 36795309Free PMC Article
Cardiac Involvement in Emery-Dreifuss Muscular Dystrophy and Related Management Strategies.
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
Coexisting Hyponatremia and Decline in Diastolic Blood Pressure Predispose to Atrial Standstill in Hyperkalemic Patients.
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
Electrophysiological Effects of Selective Atrial Coronary Artery Occlusion in Humans.
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
Dexmedetomidine-related atrial standstill and loss of capture in a pediatric patient after congenital heart surgery.
Shepard SM, Tejman-Yarden S, Khanna S, Davis CK, Batra AS
Crit Care Med 2011 Jan;39(1):187-9. doi: 10.1097/CCM.0b013e3181feb4b3. PMID: 20959781

Prognosis

Rare and potential pathogenic mutations of LMNA and LAMA4 associated with familial arrhythmogenic right ventricular cardiomyopathy/dysplasia with right ventricular heart failure, cerebral thromboembolism and hereditary electrocardiogram abnormality.
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
Electrophysiological Effects of Selective Atrial Coronary Artery Occlusion in Humans.
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
Autosomal recessive atrial dilated cardiomyopathy with standstill evolution associated with mutation of Natriuretic Peptide Precursor A.
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
Permanent atrial standstill with irregular junctional ectopic rhythm mimicking atrial fibrillation.
Türkoğlu C, Aliyev F, Celiker C, Fıratlı I
Turk Kardiyol Dern Ars 2011 Jan;39(1):55-8. PMID: 21358233
Clinical and electrophysiological characteristics of atrial standstill.
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

Cardiac and renal AL amyloidosis controlled by autologous stem cell transplantation for 17 years accompanying late onset atrial fibrillation and complete atrioventricular block.
Iijima T, Sawa N, Oba Y, Ikuma D, Mizuno H, Yamanouchi M, Suwabe T, Wake A, Kono K, Ubara Y, Ohashi K
CEN Case Rep 2023 Nov;12(4):362-367. Epub 2023 Feb 16 doi: 10.1007/s13730-023-00777-8. PMID: 36795309Free PMC Article
Rare and potential pathogenic mutations of LMNA and LAMA4 associated with familial arrhythmogenic right ventricular cardiomyopathy/dysplasia with right ventricular heart failure, cerebral thromboembolism and hereditary electrocardiogram abnormality.
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
Electrophysiological Effects of Selective Atrial Coronary Artery Occlusion in Humans.
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
Permanent atrial standstill with irregular junctional ectopic rhythm mimicking atrial fibrillation.
Türkoğlu C, Aliyev F, Celiker C, Fıratlı I
Turk Kardiyol Dern Ars 2011 Jan;39(1):55-8. PMID: 21358233
Chronic intermittent atrial standstill with intraatrial block and split atrial potentials.
Oda E, Aizawa Y, Shibuya T, Murata M, Arai Y, Ozawa T, Shibata A
Tohoku J Exp Med 1984 Aug;143(4):431-9. doi: 10.1620/tjem.143.431. PMID: 6495323

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    • ClinVar
      Related medical variations
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      Related information in NCBI Gene
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      Related information in GTR
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      Clinical tests in GTR
    • OMIM
      Related records in OMIM
    • OMIM(Genes)
      OMIM records containing genes associated with phenotypes registered in MedGen
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      Related information in PubMed Central Links
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