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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
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.
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.
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).
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.
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.
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.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

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

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
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
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
Türkoğlu C, Aliyev F, Celiker C, Fıratlı I
Turk Kardiyol Dern Ars 2011 Jan;39(1):55-8. PMID: 21358233

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
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
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
Hirosawa K, Sekiguchi M, Kasanuki H, Kimata S, Kaneko N, Nakamura K, Aosaki M, Takahashi S, Kondo M
Heart Vessels Suppl 1987;2:14-23. PMID: 3449500

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
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
Türkoğlu C, Aliyev F, Celiker C, Fıratlı I
Turk Kardiyol Dern Ars 2011 Jan;39(1):55-8. PMID: 21358233
Seino Y, Shimai S, Ibuki C, Itoh K, Takano T, Hayakawa H
J Am Coll Cardiol 1991 Aug;18(2):459-63. doi: 10.1016/0735-1097(91)90601-5. PMID: 1649852
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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