Severe cardiac conduction disturbance associated with polymyositis

1997 ◽  
Vol 7 (1) ◽  
pp. 69-74
Author(s):  
Seiji Takeda ◽  
Ichiro Tatara ◽  
Kenji Kono
1997 ◽  
Vol 7 (1) ◽  
pp. 69-74
Author(s):  
Seiji Takeda ◽  
Ichiro Tatara ◽  
Kenji Kono

Author(s):  
Taisuke Ishikawa ◽  
Hiroyuki Mishima ◽  
Julien Barc ◽  
Masanori P. Takahashi ◽  
Keiichi Hirono ◽  
...  

Background: Mutations in the nuclear envelope genes encoding LMNA and EMD are responsible for Emery-Dreifuss muscular dystrophy. However, LMNA mutations often manifest dilated cardiomyopathy with conduction disturbance without obvious skeletal myopathic complications. On the contrary, the phenotypic spectrums of EMD mutations are less clear. Our aims were to determine the prevalence of nonsyndromic forms of emerinopathy, which may underlie genetically undefined isolated cardiac conduction disturbance, and the etiology of thromboembolic complications associated with EMD mutations. Methods: Targeted exon sequencing was performed in 87 probands with familial sick sinus syndrome (n=36) and a progressive cardiac conduction defect (n=51). Results: We identified 3 X-linked recessive EMD mutations (start-loss, splicing, missense) in families with cardiac conduction disease. All 3 probands shared a common clinical phenotype of progressive atrial arrhythmias that ultimately resulted in atrial standstill associated with left ventricular noncompaction (LVNC), but they lacked early contractures and progressive muscle wasting and weakness characteristic of Emery-Dreifuss muscular dystrophy. Because the association of LVNC with EMD has never been reported, we further genetically screened 102 LVNC patients and found a frameshift EMD mutation in a boy with progressive atrial standstill and LVNC without complications of muscular dystrophy. All 6 male EMD mutation carriers of 4 families underwent pacemaker or defibrillator implantation, whereas 2 female carriers were asymptomatic. Notably, a strong family history of stroke observed in these families was probably due to the increased risk of thromboembolism attributable to both atrial standstill and LVNC. Conclusions: Cardiac emerinopathy is a novel nonsyndromic X-linked progressive atrial standstill associated with LVNC and increased risk of thromboembolism.


Cureus ◽  
2021 ◽  
Author(s):  
Mohamed Elhassan ◽  
Hasan Ahmad ◽  
Mohamed Mohamed ◽  
Ola Saidahmed ◽  
Ahmed E Elhassan

2008 ◽  
Vol 152 (1) ◽  
pp. 85-89 ◽  
Author(s):  
Shuenn-Nan Chiu ◽  
Jou-Kou Wang ◽  
Mei-Hwan Wu ◽  
Chi-Wei Chang ◽  
Chun-An Chen ◽  
...  

2010 ◽  
Vol 6 (1) ◽  
pp. 27-30 ◽  
Author(s):  
Emmanuel M. Bhaskar ◽  
Swathy Moorthy ◽  
Gaurav Ganeshwala ◽  
Georgi Abraham

PEDIATRICS ◽  
1973 ◽  
Vol 51 (6) ◽  
pp. 1083-1085
Author(s):  
Thomas W. Rowland ◽  
Mohsen Gharib

Significant cardiac conduction disturbance in patients with central nervous system disease rarely occurs. While commonly induced in the experimental animal the appearance of such arrhythmias in the human has been limited to single reports of ectopic tachycardia and atrioventricular (AV) block with several CNS disorders.1-5 This article presents a child with cyanotic congenital heart disease who developed persistent AV block following needle aspiration of a right thalamic brain abscess. Case Report L.D. is a 9-year-old girl with tetralogy of Fallot for which she has undergone three systemic-pulmonary arterial anastomoses. Following the last of these, a Waterston shunt in 1965, she had done well on digoxin, .090 mg twice a day with minimal exercise intolerance and cyanosis.


2011 ◽  
Vol 1 (2) ◽  
pp. 43-46
Author(s):  
Maysara M. Abdelaziz ◽  
Anand N. Kamalanathan ◽  
Cheri Mathews John

Holiday heart syndrome is an acute cardiac rhythm or conduction disturbance associated with heavy ethanol consumption in a person without other clinical evidence of heart disease. Typically, this resolves rapidly with spontaneous recovery during subsequent abstinence from alcohol use. Two adolescents were presented with different degrees of heart block following acute alcohol ingestion. The electrocardiograph abnormalities resolved within 12 hours. Further investigations, including echocardiography and 24-hour electrocardiograph, were normal. Emergency Department physicians should be aware of the possibility of acute alcohol ingestion causing changes in cardiac conduction which has a potential to develop into life-threatening arrhythmias, thus, can occur in children and young adolescents.


2011 ◽  
Vol 1 (2) ◽  
pp. 43-46
Author(s):  
Maysara M. Abdelaziz ◽  
Anand N. Kamalanathan ◽  
Cheri Mathews John

Holiday heart syndrome is an acute cardiac rhythm or conduction disturbance associated with heavy ethanol consumption in a person without other clinical evidence of heart disease. Typically, this resolves rapidly with spontaneous recovery during subsequent abstinence from alcohol use. Two adolescents were presented with different degrees of heart block following acute alcohol ingestion. The electrocardiograph abnormalities resolved within 12 hours. Further investigations, including echocardiography and 24-hour electrocardiograph, were normal. Emergency Department physicians should be aware of the possibility of acute alcohol ingestion causing changes in cardiac conduction which has a potential to develop into life-threatening arrhythmias, thus, can occur in children and young adolescents.


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