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British Heart Journal Apr 1988Three of nine siblings were affected by atrial standstill. Clinical, electrophysiological, and histological findings in two of these cases were studied....
Three of nine siblings were affected by atrial standstill. Clinical, electrophysiological, and histological findings in two of these cases were studied. Electrophysiological studies showed persistent atrial standstill in one and partial atrial standstill in the other. Biopsy specimens of the right atria showed amyloid deposits, but specimens of the right ventricles and skin did not. Neither patient had peripheral neuropathy, gastrointestinal disorder, or renal dysfunction. Familial atrial standstill is usually associated with peripheral neuropathy. In the two cases studied amyloid deposition mainly affected the atrium, rather than the ventricles or other organs, and caused atrial standstill.
Topics: Adult; Amyloidosis; Arrhythmias, Cardiac; Cardiomyopathies; Heart Atria; Humans; Male; Middle Aged
PubMed: 3370185
DOI: 10.1136/hrt.59.4.498 -
Japanese Circulation Journal Jul 2000Two cases of atrial standstill are presented, one with cardiac amyloidosis, the other with idiopathic dilated cardiomyopathy. The plasma atrial natriuretic peptide (ANP)...
Two cases of atrial standstill are presented, one with cardiac amyloidosis, the other with idiopathic dilated cardiomyopathy. The plasma atrial natriuretic peptide (ANP) concentration was normal to slightly elevated in both patients, despite a marked elevation of the plasma brain natriuretic peptide (BNP) concentration. In the patient with amyloidosis (ANP: 170 pg/ml, BNP: 1220 pg/ml), a dual chamber pacemaker was successfully implanted for the treatment of sinus arrest. However, loss of atrial capture occurred 1 month later. In the patient with dilated cardiomyopathy (ANP: 47 pg/ml, BNP: 422 pg/ml), an electrophysiologic study confirmed persistent atrial standstill and failure to pace from either the right atrium or the coronary sinus. The hypothesis is that the attenuated increase in plasma ANP concentration relative to the increase in the BNP concentration may be a sensitive and useful marker to confirm atrial standstill in the setting of congestive heart failure.
Topics: Atrial Function; Atrial Natriuretic Factor; Biomarkers; Electrocardiography; Heart Atria; Heart Failure; Humans; Male; Middle Aged; Prognosis
PubMed: 10929785
DOI: 10.1253/jcj.64.537 -
Heart (British Cardiac Society) Oct 2003
Topics: Aged; Aged, 80 and over; Anticoagulants; Atrial Function, Left; Catheterization; Echocardiography; Echocardiography, Transesophageal; Electrocardiography; Heart Block; Heart Valve Prosthesis Implantation; Humans; Male; Mitral Valve Stenosis; Risk Factors; Thrombosis; Warfarin
PubMed: 12975410
DOI: 10.1136/heart.89.10.1173 -
Journal of Korean Medical Science Apr 2005Emery-Dreifuss muscular dystrophy (EDMD) and limb-girdle muscular dystrophy type 1B (LGMD1B) are characterized by cardiac dysrhythmias, late-onset cardiomyopathy, slowly...
Emery-Dreifuss muscular dystrophy (EDMD) and limb-girdle muscular dystrophy type 1B (LGMD1B) are characterized by cardiac dysrhythmias, late-onset cardiomyopathy, slowly progressive skeletal myopathy and contractures of the neck, elbows and ankles. The causative mutation is either in the emerin gene (X-linked recessive EDMD) or lamin A/C gene (autosomal dominant EDMD2 or LGMD1B). We report three cases of EDMD, EDMD2 and LGMD1B. A 14-yr-old boy showed limitation of cervical flexion and contractures of both elbows and ankles. Sinus arrest with junctional escape beats was noted. He was diagnosed as X-linked recessive EDMD (MIM 310300). A 28-yr-old female showed severe wasting and weakness of humeroperoneal muscles. Marked limitation of cervical flexion and contractures of both elbows and ankles were noted. Varying degrees of AV block were noted. She was diagnosed as autosomal dominant EDMD2 (MIM 181350). A 41-yr-old female had contractures of both ankles and limb-girdle type muscular dystrophy. ECG revealed atrial tachycardia with high grade AV block. She was diagnosed as autosomal dominant LGMD1B (MIM 159001). Cardiac dysrhythmias in EDMD and LGMD1B include AV block, bradycardia, atrial tachycardia, atrial fibrillation, and atrial standstill, causing sudden death necessitating pacemaker implantation. Cardiologists should know about these unusual genetic diseases with conduction defects, especially in young adults.
Topics: Adolescent; Adult; Arrhythmias, Cardiac; Cardiomyopathies; Female; Humans; Male; Muscle, Skeletal; Muscular Dystrophies, Limb-Girdle; Muscular Dystrophy, Emery-Dreifuss
PubMed: 15832002
DOI: 10.3346/jkms.2005.20.2.283 -
Journal of Cardiology Cases May 2018The long-term effects of enzyme replacement therapy (ERT) on cardiac function and the conduction system in Fabry disease are not clearly understood. We report a case of...
The long-term effects of enzyme replacement therapy (ERT) on cardiac function and the conduction system in Fabry disease are not clearly understood. We report a case of a 48-year-old man with non-classical Fabry disease treated with ERT for 11 years. He was diagnosed with Fabry disease at age 27 years based on the presence of decreased alpha-galactosidase A activity in the peripheral leukocytes and of the causal alpha-galactosidase A mutation (Val339Gln). Subsequently, peritoneal dialysis was initiated for renal failure at age 35 years. ERT was initiated at age 39 years to halt the progression of cardiac dysfunction. Electrical conduction disturbances progressed gradually to complete atrioventricular block with atrial standstill during 9 years of ERT despite the lack of progression of ventricular hypertrophy. Although he underwent permanent pacemaker implantation to prevent sudden cardiac death, the atrioventricular junctional rhythm remained, thereby lowering the ventricular pacing rate. Based on this case, we recognize that the effects of ERT are limited for inhibiting the progression of Fabry disease and especially for inhibiting arrhythmia and conduction disturbances. Early diagnosis of Fabry disease and early initiation of ERT might be the key to further improvements in this disease and its associated conditions. < We encountered a patient with Fabry disease treated with long-term enzyme replacement therapy (ERT) in whom conduction disturbances progressed without progression of left ventricular hypertrophy. This case suggests that ERT is limited for inhibiting the progression of Fabry disease and especially of arrhythmia and conduction disturbances. Early diagnosis of Fabry disease and initiation of ERT may be important for providing further improvements of this condition.>.
PubMed: 30279886
DOI: 10.1016/j.jccase.2018.01.004 -
Compound Heterozygous Mutations in Severe Sodium Channelopathy With Brugada Syndrome: A Case Report.Frontiers in Cardiovascular Medicine 2020Brugada syndrome (BrS) is an inherited cardiac arrhythmia with an increased risk for sudden cardiac death (SCD). About 20% of BrS cases are explained by mutations in...
Brugada syndrome (BrS) is an inherited cardiac arrhythmia with an increased risk for sudden cardiac death (SCD). About 20% of BrS cases are explained by mutations in the gene, encoding the main cardiac sodium Na1.5 channel. Here we present a severe case of cardiac sodium channelopathy with BrS caused by compound heterozygous mutations. We performed a genetic analysis of in a male proband who collapsed during cycling at the age of 2 years. Because of atrial standstill, he received a pacemaker, and at the age of 3 years, he experienced a collapse anew with left-sided brain stroke. A later ECG taken during a fever unmasked a characteristic BrS type-1 pattern. The functional effect of the detected genetic variants was investigated. Next-generation sequencing allowed the detection of two variants in : c.4813+3_4813+6dupGGGT-a Belgian founder mutation-and c.4711 T>C, p.Phe1571Leu. A familial segregation analysis showed the presence of the founder mutation in the proband's affected father and paternal aunt and the occurrence of the p.Phe1571Leu. The functional effect of the founder mutation was previously described as a loss-of-function. We performed a functional analysis of the p.Phe571Leu variant in HEK293 cells alone or co-expressed with the β-subunit. Compared to the wild type, p.Phe1571Leu displayed a hyperpolarizing shift in the voltage dependence of inactivation (loss-of-function), while the activation parameters were unaffected. Using the peptide toxin nemertide α-1, the variant's loss-of-function effect could be restored due to a toxin-dependent reduction of channel inactivation. This is the first report providing support for the pathogenicity of the p.Phe1571Leu variant which, together with the c.4813+3_4813+6dupGGGT founder mutation, explains the severity of the phenotype of cardiac sodium channelopathy with BrS in the presented case.
PubMed: 32850980
DOI: 10.3389/fcvm.2020.00117 -
HeartRhythm Case Reports Nov 2017
PubMed: 29387541
DOI: 10.1016/j.hrcr.2017.07.014 -
Medicine Feb 2024Previous studies have found that the main treatment of sinus arrest is pacemaker treatment. It is rare to have 12 s of sinus arrest after radiofrequency ablation, and...
RATIONALE
Previous studies have found that the main treatment of sinus arrest is pacemaker treatment. It is rare to have 12 s of sinus arrest after radiofrequency ablation, and whether a permanent pacemaker is implanted immediately in this case is not described in the guidelines.
PATIENT CONCERNS
A 76-year-old male patient with persistent atrial fibrillation (AF) developed sinus arrest lasting 12 s in the early morning of the fourth day after using radiofrequency ablation for pulmonary vein isolation.
DIAGNOSIS
The patient was diagnosed with AF and sinus arrest.
INTERVENTIONS
The patient received cardiopulmonary resuscitation, intravenous injection of atropine 1 mg, and intravenous infusion of isoproterenol 1mg and immediately recovered consciousness thereafter. Approximately, 1.5 h later, the patient underwent surgery to install a temporary pacemaker in the right femoral vein.
OUTCOMES
The patient had repeated episodes of sinus arrest after the implantation of a temporary pacemaker. After 3 weeks, the patient stabilized and was discharged. The patient was followed up for 1 year and did not experience any recurrence of sinus arrest or AF.
LESSONS
We consider the potential for postoperative myocardial edema, injury to the sinoatrial node during the procedure, propafenone poisoning, and autonomic dysfunction as contributors to the occurrence of sinus arrest after radiofrequency ablation. When sinus arrest occurs after radiofrequency ablation, we can choose the appropriate treatment according to the patient's condition.
Topics: Male; Humans; Aged; Treatment Outcome; Catheter Ablation; Atrial Fibrillation; Radiofrequency Ablation; Heart Arrest; Heart Atria; Heart Block; Cardiomyopathies; Genetic Diseases, Inborn
PubMed: 38394505
DOI: 10.1097/MD.0000000000037191 -
British Heart Journal May 1962
Topics: Arrhythmias, Cardiac; Brugada Syndrome; Cardiac Conduction System Disease; Cardiomyopathies; Genetic Diseases, Inborn; Heart Atria; Heart Block; Heart Conduction System; Humans; Hyperkalemia; Nephritis; Potassium
PubMed: 13923225
DOI: 10.1136/hrt.24.3.389 -
Yonsei Medical Journal Feb 2009We report a 55-year-old female patient who presented with no P waves but with a wide QRS complex escape rhythm at 44 beats/min and prolonged QTc of 0.55 seconds on ECG....
We report a 55-year-old female patient who presented with no P waves but with a wide QRS complex escape rhythm at 44 beats/min and prolonged QTc of 0.55 seconds on ECG. The patient had recurrence of ventricular fibrillations and loss of consciousness, and underwent defibrillation and cardiopulmonary resuscitation (CPR) several times because of cardiac arrest. The transthoracic echocardiography showed dilated cardiomyopathy and enlargement of both atria. The Doppler echocardiography documented the absence of A wave in the tricuspid and mitral valve flow. An electrophysiologic study demonstrated electrical inactivity in the right and left atria. Atrial pacing with maximum output did not capture the atria. These findings together with her electrocardiographic finding indicated atrial standstill. Sudden cardiac death was her first clinical manifestation of ventricular arrhythmia. The patient remained asymptomatic after receiving a single chamber implantable cardioverter-defibrillator (ICD) with VVI pacemaker function.
Topics: Bradycardia; Cardiomyopathy, Dilated; Death, Sudden, Cardiac; Defibrillators, Implantable; Electrocardiography; Female; Heart Atria; Humans; Middle Aged; Ventricular Fibrillation
PubMed: 19259364
DOI: 10.3349/ymj.2009.50.1.156