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Kokyu To Junkan. Respiration &... Apr 1988
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Acta Cardiologica 1975The authors present a case of permanent atrial standstill with syncopal attacks, in a patient with chronic Chagas' Heart Disease. The recognition of this dysrhythmia was...
The authors present a case of permanent atrial standstill with syncopal attacks, in a patient with chronic Chagas' Heart Disease. The recognition of this dysrhythmia was based upon the conventional and intracavity electrocardiographic tracings in addition to phonomecanographic and hemodynamic data. The recording of the His Bundle electrogram demonstrated the absence of atrial activity, with the His potential preceding all ventricular complexes and an advanced conduction defect distal to the bundle of His. A diffuse type of atrial involvement was suggested by the lack of response to pacemaker stimulation. An increase in ventricular rate following intravenous atropine administration, led to the diagnosis of an a-v junctional rhythm with a widened QRS complex due to an associated right bundle branch block. Following the implantation of an epicardial ventricular pacemaker, the patient became completely asymptomatic despite the persistence of electrical and mechanical atrial standstill.
Topics: Adult; Arrhythmias, Cardiac; Bundle of His; Heart Atria; Heart Conduction System; Humans; Male; Pacemaker, Artificial
PubMed: 1084660
DOI: No ID Found -
The Journal of Tehran Heart Center 2011We introduce a 32-year-old man who was evaluated for a dizziness and headache of unknown origin for at least two months and was referred to our center after ECG...
We introduce a 32-year-old man who was evaluated for a dizziness and headache of unknown origin for at least two months and was referred to our center after ECG findings. He was finally diagnosed as a case of idiopathic, familial, diffuse, persistent atrial standstill, which is a rare arrhythmogenic condition characterized by the absence of electrical and mechanical activity in the atria. He successfully received a single-chamber permanent pacemaker.
PubMed: 23074623
DOI: No ID Found -
Circulation Apr 1973
Topics: Atrial Fibrillation; Digitalis Glycosides; Heart Atria; Humans
PubMed: 4696806
DOI: 10.1161/01.cir.47.4.913-b -
Journal of Veterinary Cardiology : the... Jun 2017Persistent atrial standstill is a rare arrhythmia in both human and veterinary patients. In recent decades, cases of partial atrial standstill have been recognized in...
Persistent atrial standstill is a rare arrhythmia in both human and veterinary patients. In recent decades, cases of partial atrial standstill have been recognized in humans. We describe a case of presumptive partial atrial standstill in a Greyhound, in which there was disparate left and right atrial electromechanical function and rapid progression to congestive heart failure over the span of fourteen weeks. An atrial cardiomyopathy characterized by severe, diffuse, fibrofatty replacement of the atrial myocardium was identified histologically.
Topics: Animals; Arrhythmias, Cardiac; Cardiomyopathies; Dog Diseases; Dogs; Electrocardiography; Fatal Outcome; Female; Genetic Diseases, Inborn; Heart Atria; Heart Block
PubMed: 28314614
DOI: 10.1016/j.jvc.2017.01.003 -
Pacing and Clinical Electrophysiology :... May 2018Atrial standstill is an arrhythmogenic condition characterized by the absence of spontaneous electrical and mechanical atrial activity or in response to stimulation....
BACKGROUND
Atrial standstill is an arrhythmogenic condition characterized by the absence of spontaneous electrical and mechanical atrial activity or in response to stimulation. There are few reported familial cases which have been associated with SCN5A mutations cosegregating with GJA5 or RYR2; however, isolated SCN5A mutations are rare.
OBJECTIVE
The purpose of this study was to determine the clinical and biophysical consequence of a novel SCN5A mutation identified in a family with progressive atrial standstill and sudden death.
METHODS
The family of a sporadic case of congenital atrial standstill underwent genetic screening. Human Embryonic Kidney 293 cells were transfected with wild-type (WT) or mutant SCN5A cDNAs. Biophysical properties were studied using whole-cell using patch clamp methods.
RESULTS
A novel homozygous SCN5A mutation, p.V1340L, was identified in the proband and her sister. The proband had complete atrial standstill whereas the sister had partial atrial standstill. Heterozygous mutations were identified in the mother, father, and brother. All three had normal sinus rhythm and were asymptomatic. The mutant Nav1.5(V1340L) reduced Nav1.5 current density as well as showed a depolarizing shift in the voltage-dependent steady-state activation (WT: -35.3 ± 1.62 mV; V1340L: -22.4 ± 2.59 mV; P = 0.001).
CONCLUSIONS
A homozygous loss-of-function SCN5A mutation likely results in atrial standstill and sudden death due to suppression of initiation of action potential.
PubMed: 29781517
DOI: 10.1111/pace.13386 -
Journal of the American Society of... Jul 2020
Topics: Atrial Fibrillation; Cardiomyopathies; Cardiomyopathy, Hypertrophic; Genetic Diseases, Inborn; Heart Atria; Heart Block; Humans
PubMed: 32305175
DOI: 10.1016/j.echo.2020.02.011 -
Indian Pediatrics Feb 2016Atrial standstill manifests as absence of any atrial electrical activity in the surface ECG leads. Persistent atrial standstill secondary to acute myocarditis is...
BACKGROUND
Atrial standstill manifests as absence of any atrial electrical activity in the surface ECG leads. Persistent atrial standstill secondary to acute myocarditis is extremely rare.
CASE REPORT
10-year-old girl had atrial standstill and heart failure due to acute myocarditis. After recovery from myocarditis, heart failure resolved, but the atrial standstill persisted.
OUTCOME
Persistent atrial standstill was treated with permanent pacemaker and anticoagulation.
MESSAGE
Acute myocarditis may rarely cause atrial standstill that can last even after recovery from myocarditis.
Topics: Arrhythmias, Cardiac; Child; Echocardiography, Doppler; Female; Heart Atria; Heart Failure; Humans; Myocarditis; Pacemaker, Artificial
PubMed: 26897154
DOI: 10.1007/s13312-016-0814-3 -
Circulation. Cardiovascular Genetics Feb 2013Atrial dilatation and atrial standstill are etiologically heterogeneous phenotypes with poorly defined nosology. In 1983, we described 8-years follow-up of atrial...
BACKGROUND
Atrial dilatation and atrial standstill are etiologically heterogeneous phenotypes with poorly defined nosology. In 1983, we described 8-years follow-up of atrial dilatation with standstill evolution in 8 patients from 3 families. We later identified 5 additional patients with identical phenotypes: 1 member of the largest original family and 4 unrelated to the 3 original families. All families are from the same geographic area in Northeast Italy.
METHODS AND RESULTS
We followed up the 13 patients for up to 37 years, extended the clinical investigation and monitoring to living relatives, and investigated the genetic basis of the disease. The disease was characterized by: (1) clinical onset in adulthood; (2) biatrial dilatation up to giant size; (3) early supraventricular arrhythmias with progressive loss of atrial electric activity to atrial standstill; (4) thromboembolic complications; and (5) stable, normal left ventricular function and New York Heart Association functional class during the long-term course of the disease. By linkage analysis, we mapped a locus at 1p36.22 containing the Natriuretic Peptide Precursor A gene. By sequencing Natriuretic Peptide Precursor A, we identified a homozygous missense mutation (p.Arg150Gln) in all living affected individuals of the 6 families. All patients showed low serum levels of atrial natriuretic peptide. Heterozygous mutation carriers were healthy and demonstrated normal levels of atrial natriuretic peptide.
CONCLUSIONS
Autosomal recessive atrial dilated cardiomyopathy is a rare disease associated with homozygous mutation of the Natriuretic Peptide Precursor A gene and characterized by extreme atrial dilatation with standstill evolution, thromboembolic risk, preserved left ventricular function, and severely decreased levels of atrial natriuretic peptide.
Topics: Adult; Atrial Natriuretic Factor; Base Sequence; Cardiomyopathies; Cardiomyopathy, Dilated; Cohort Studies; Female; Follow-Up Studies; Genetic Diseases, Inborn; Heart Atria; Heart Block; Humans; Italy; Male; Middle Aged; Molecular Sequence Data; Mutation; Pedigree; Phenotype
PubMed: 23275345
DOI: 10.1161/CIRCGENETICS.112.963520 -
HeartRhythm Case Reports Nov 2017
PubMed: 29387541
DOI: 10.1016/j.hrcr.2017.07.014