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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 Internal Medicine Jan 2004Mutations in the cardiac Na+ channel gene SCN5A are responsible for multiple lethal ventricular arrhythmias including Brugada syndrome and congenital long QT syndrome....
Mutations in the cardiac Na+ channel gene SCN5A are responsible for multiple lethal ventricular arrhythmias including Brugada syndrome and congenital long QT syndrome. Here we report a case of Brugada syndrome with ST elevation in the right precordial and inferior leads accompanied by atrial standstill and spontaneous ventricular fibrillation. Atrial standstill and J wave elevation were provoked by procainamide. Genetic analysis revealed a missense mutation (R367H) in SCN5A. The resultant mutant Na+ channel was nonfunctional when expressed heterologously in Xenopus oocytes. Our study suggests that genetic defects in SCN5A may be associated with atrial standstill in combination with ventricular arrhythmias.
Topics: Adult; Electrocardiography; Female; Heart; Heart Atria; Heart Function Tests; Humans; Mutation, Missense; Myocardium; NAV1.5 Voltage-Gated Sodium Channel; Polymerase Chain Reaction; Sodium Channels; Syndrome; Ventricular Fibrillation
PubMed: 14687250
DOI: 10.1046/j.0954-6820.2003.01247.x -
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 -
Circulation Journal : Official Journal... Dec 2002A 59-year-old woman with partial atrial standstill was studied using magnetocardiograms (MCGs), which revealed through QRS-T subtraction and time-frequency analysis that...
A 59-year-old woman with partial atrial standstill was studied using magnetocardiograms (MCGs), which revealed through QRS-T subtraction and time-frequency analysis that there was a high-frequency (6 Hz) magnetic source at the low atrial septum. MCGs are useful for noninvasively evaluating the clinical course of patients with atrial fibrillation.
Topics: Algorithms; Atrial Fibrillation; Atrial Function; Electrocardiography; Female; Heart Arrest; Heart Septum; Humans; Magnetics; Middle Aged; Subtraction Technique
PubMed: 12499629
DOI: 10.1253/circj.66.1178 -
The Canadian Veterinary Journal = La... Oct 2002An Ibizan hound cross was referred with a 1-year history of syncope and exercise intolerance. An electrocardiogram showed bradycardia and no P waves; atrial standstill...
An Ibizan hound cross was referred with a 1-year history of syncope and exercise intolerance. An electrocardiogram showed bradycardia and no P waves; atrial standstill was diagnosed. A permanent artificial endocardial pacemaker was implanted. The lead dislodged and was reimplanted into the right ventricular apex. Capture was once again achieved.
Topics: Animals; Bradycardia; Cardiac Pacing, Artificial; Dog Diseases; Dogs; Electrocardiography; Female; Pacemaker, Artificial
PubMed: 12395764
DOI: No ID Found -
Nihon Ronen Igakkai Zasshi. Japanese... Jul 2001We describe an elderly case of idiopathic dilatation of the right atrium in which right-sided heart failure was exacerbated by drug-induced bradyarrhythmia. An...
We describe an elderly case of idiopathic dilatation of the right atrium in which right-sided heart failure was exacerbated by drug-induced bradyarrhythmia. An 84-year-old man, who had a 10-year history of episodic edema, was treated with proscillaridin and verapamil hydrochloride at another hospital. He had experienced a poor appetite and general malaise 2 months previously, and exertional dyspnea 10 days previously. On admission, he had jugular venous dilatation, systemic edema, and hepatomegaly. On auscultation, a third heart sound originating from the right ventricle and systolic murmur of tricuspid regurgitation were heard. An admission electrocardiogram showed an atrial standstill and junctional escape rhythm with a QRS rate of 31 beats/minute. Chest roentgenogram revealed a bilateral pleural effusion and cardiomegaly with a cardiothoracic ratio of 76%, but no pulmonary congestion. Echocardiogram disclosed idiopathic dilatation of the right atrium and secondary tricuspid regurgitation. He was given a diagnosis of right-sided heart failure due to idiopathic dilatation of the right atrium exacerbated by bradyarrhythmia, which was suspected to derive from the side effects of proscillaridin and verapamil hydrochloride. Thus, these agents were withheld. In addition, the patient reduced sodium intake and was treated with diuretics and beta-adrenergic agonist. Thereafter, right-sided heart failure markedly improved. At the time of the last follow-up 16 months after discharge, he felt well.
Topics: Aged; Aged, 80 and over; Atrial Fibrillation; Bradycardia; Cardiomegaly; Cardiotonic Agents; Dilatation, Pathologic; Heart Atria; Heart Failure; Humans; Male; Proscillaridin; Verapamil
PubMed: 11523170
DOI: 10.3143/geriatrics.38.544 -
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 -
Revista Espanola de Cardiologia Dec 1999Atrial standstill is a very rare form of bradyarrhythmia and consists of a transitory or permanent loss of the electrical and mechanical activity of the atria. We report...
Atrial standstill is a very rare form of bradyarrhythmia and consists of a transitory or permanent loss of the electrical and mechanical activity of the atria. We report a series of 8 patients, all of them with rheumatic valve disease (5 of them with a prosthetic valve) with symptomatic bradyarrhythmia secondary to atrial standstill, requiring an implantable pacemaker.
Topics: Aged; Bradycardia; Female; Heart Atria; Heart Valve Diseases; Heart Valve Prosthesis; Humans; Male; Middle Aged; Mitral Valve; Pacemaker, Artificial; Rheumatic Heart Disease
PubMed: 10659665
DOI: No ID Found -
Nihon Ronen Igakkai Zasshi. Japanese... Jan 1999We report an autopsy case of an 88-year-old man with idiopathic enlargement of the right atrium which is considered to be the oldest case reported. The patient was given... (Review)
Review
We report an autopsy case of an 88-year-old man with idiopathic enlargement of the right atrium which is considered to be the oldest case reported. The patient was given a diagnosis of atrial fibrillation at the age of 75 years, when he developed congestive heart failure. Bradycardia associated with partial atrial standstill was detected and, the patient underwent implantation of a pacemaker at age 77. An echocardiogram revealed marked enlargement of the right atrium and moderate enlargement of the left atrium. Thus, idiopathic enlargement of the right atrium was diagnosed. He had recurrent congestive heart failure before admission to our hospital because of malnutrition and anemia. Although he was treated with high calorie intravenous infusion and blood transfusion, he died of pneumonia and heart failure. Postmortem examination revealed that the heart weighed 430 g, and there was marked dilatation of the right atrium which had an extremely thin wall. The annular circumference of the tricuspid valve was markedly dilated, 170 mm, resulting in tricuspid regurgitation. The left atrium was moderately dilated and the right and left ventricles were slightly dilated. Histologically, the free wall of the right atrium was totally replaced by fibrous tissue and atrioventricular valves did not reveal any rheumatic changes. These pathological findings were compatible with idiopathic enlargement of the right atrium. There has been no previous case report of idiopathic enlargement of the right atrium in a patient aged 80 years of age or over.
Topics: Aged; Aged, 80 and over; Cardiomegaly; Heart Atria; Humans; Male
PubMed: 10332196
DOI: 10.3143/geriatrics.36.59 -
Japanese Circulation Journal Jun 1996Acute infectious myocarditis is primarily by viruses and bacteria, but sometimes by rickettsia. Tsutsugamushi disease is a febrile illness caused by Rickettsia...
Acute infectious myocarditis is primarily by viruses and bacteria, but sometimes by rickettsia. Tsutsugamushi disease is a febrile illness caused by Rickettsia tsutsugamushi, and has been prevalent in Korea since 1985. Characteristics of tsutsugamushi disease are fever, rash and eschar. Tsutsugamushi myocarditis is rare. Cardiac involvement may include ST-T changes, PR prolongation, mild mitral regurgitation, and perivascular inflammation with myocardial necrosis. We describe here a 50-year-old woman who complained of fever, orthopnea and chest pain. Work-up of the patient revealed abdominal scar, positive tsutsugamushi antibody, congestive heart failure with severe mitral and tricuspid regurgitation, persistent atrial standstill on electrophysiologic study, junctional rhythm and ST-T changes mimicking anterior myocardial infarction and myocardial inflammation with perivasculitis on endomyocardial biopsy. The patient's condition improved with doxycycline and inotropics. Persistent atrial standstill during was found at the one-year follow-up.
Topics: Arrhythmias, Cardiac; Atrial Function; Female; Heart Atria; Heart Conduction System; Heart Failure; Humans; Middle Aged; Myocarditis
PubMed: 8844306
DOI: 10.1253/jcj.60.382