U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Atrial standstill 2(ATRST2)

MedGen UID:
816731
Concept ID:
C3810401
Disease or Syndrome
Synonyms: ATRIAL DILATION AND STANDSTILL; ATRST2; CARDIOMYOPATHY, ATRIAL DILATED, WITH ATRIAL STANDSTILL
 
Gene (location): NPPA (1p36.22)
 
Monarch Initiative: MONDO:0014329
OMIM®: 615745

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). For a discussion of genetic heterogeneity of atrial standstill, see ATRST1 (108770). [from OMIM]

Clinical features

From HPO
Vascular dilatation
MedGen UID:
8076
Concept ID:
C0002940
Pathologic Function
Abnormal outpouching or sac-like dilatation in the wall of an atery, vein or the heart.
Palpitations
MedGen UID:
14579
Concept ID:
C0030252
Finding
A sensation that the heart is pounding or racing, which is a non-specific sign but may be a manifestation of arrhythmia.
Stroke disorder
MedGen UID:
52522
Concept ID:
C0038454
Disease or Syndrome
Sudden impairment of blood flow to a part of the brain due to occlusion or rupture of an artery to the brain.
Atrial arrhythmia
MedGen UID:
39317
Concept ID:
C0085611
Pathologic Function
A type of supraventricular tachycardia in which the atria are the principal site of electrophysiologic disturbance.
Bradycardia
MedGen UID:
140901
Concept ID:
C0428977
Finding
A slower than normal heart rate (in adults, slower than 60 beats per minute).
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.
Cardiomyopathy
MedGen UID:
209232
Concept ID:
C0878544
Disease or Syndrome
A myocardial disorder in which the heart muscle is structurally and functionally abnormal, in the absence of coronary artery disease, hypertension, valvular disease and congenital heart disease sufficient to cause the observed myocardial abnormality.
Absent P wave
MedGen UID:
324892
Concept ID:
C1837847
Finding
The P wave that normally precedes each QRS complex by a fixed PR interval of 120 to 200 milliseconds is not present.
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.
Dilatation of the ventricular cavity
MedGen UID:
1393082
Concept ID:
C4476545
Anatomical Abnormality
A localized outpouching of ventricular cavity that is generally associated with dyskinesia and paradoxical expansion during systole.
Scarring
MedGen UID:
3093
Concept ID:
C0008767
Pathologic Function
A scar refers to a lesion in which wound, burn, or sore has not healed completely and fibrous connective tissue has developed.
Dyspnea
MedGen UID:
3938
Concept ID:
C0013404
Sign or Symptom
Difficult or labored breathing. Dyspnea is a subjective feeling only the patient can rate, e.g., on a Borg scale.
Hyperpepsinogenemia I
MedGen UID:
871164
Concept ID:
C4025638
Disease or Syndrome

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

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
Alkorashy M, Al-Ghamdi B, Tulbah S, Al-Numair NS, Alhadeq F, A Takroni S, Al-Hassnan ZN
Pacing Clin Electrophysiol 2021 Feb;44(2):380-384. Epub 2020 Oct 1 doi: 10.1111/pace.14077. PMID: 32965045
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
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
Bensaid J
Am Heart J 1996 Feb;131(2):404-7. doi: 10.1016/s0002-8703(96)90376-2. PMID: 8579043

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
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
Bensaid J
Am Heart J 1996 Feb;131(2):404-7. doi: 10.1016/s0002-8703(96)90376-2. PMID: 8579043

Therapy

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
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
Takehara N, Kawamura Y, Sato N, Sato A, Kakuchi H, Kikuchi K
J Electrocardiol 2001 Oct;34(4):339-44. doi: 10.1054/jelc.2001.27840. PMID: 11590576
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

Alkorashy M, Al-Ghamdi B, Tulbah S, Al-Numair NS, Alhadeq F, A Takroni S, Al-Hassnan ZN
Pacing Clin Electrophysiol 2021 Feb;44(2):380-384. Epub 2020 Oct 1 doi: 10.1111/pace.14077. PMID: 32965045
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
Bensaid J
Am Heart J 1996 Feb;131(2):404-7. doi: 10.1016/s0002-8703(96)90376-2. PMID: 8579043
Talwar KK, Radhakrishnan S, Chopra P
Int J Cardiol 1988 Aug;20(2):283-6. doi: 10.1016/0167-5273(88)90275-6. PMID: 3209261

Clinical prediction guides

Alkorashy M, Al-Ghamdi B, Tulbah S, Al-Numair NS, Alhadeq F, A Takroni S, Al-Hassnan ZN
Pacing Clin Electrophysiol 2021 Feb;44(2):380-384. Epub 2020 Oct 1 doi: 10.1111/pace.14077. PMID: 32965045
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
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
Messinger WJ, Mirkinson AM
Arch Intern Med 1969 Aug;124(2):211-4. doi: 10.1001/archinte.124.2.211. PMID: 5797081