Progression of Juvenile Atrial Standstill in a Boy with SCN5A Mutation

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منابع مشابه

Congenital atrial standstill associated with coinheritance of a novel SCN5A mutation and connexin 40 polymorphisms.

BACKGROUND Congenital atrial standstill has been linked to SCN5A. Incomplete penetrance observed in atrial standstill has been attributed in part to the digenic inheritance of polymorphisms in the atrial-specific gap junction connexin 40 (Cx40) in conjunction with an SCN5A mutation. OBJECTIVES The purpose of this study was to determine the clinical and biophysical characteristics of a novel S...

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Temporary atrial standstill in a crossbred dog associated with bladder outlet obstruction

A 6-year-old male crossbred dog was referred to the Veterinary Medical Teaching Hospital of Islamic Azad University of Karaj with the history of abdominal distention, rectal tenesmus and stranguria. Abdominal radiographs and ultrasonography indicated large distended urinary bladder and prostatic enlargement. ECG showed temporary episodes of atrial standstill. The only abnormalities detected by ...

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A cardiac sodium channel mutation cosegregates with a rare connexin40 genotype in familial atrial standstill.

Atrial standstill (AS) is a rare arrhythmia that occasionally appears to be genetically determined. This study investigates the genetic background of this arrhythmogenic disorder in a large family. Forty-four family members were clinically evaluated. One deceased and three living relatives were unambiguously affected by AS. All other relatives appeared unaffected. Candidate gene screening revea...

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Atrial standstill

A 37-year-old male patient was referred to our cardiology division with a longstanding and worsening history of short episodes of dizziness and reduced effort tolerance. He denied any syncope, palpitations or chest pain. His medical history was unremarkable and he took no medication. Family history was negative for cardiac diseases at a young age or premature sudden death. On clinical examinati...

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Autosomal recessive atrial dilated cardiomyopathy with standstill evolution associated with mutation of Natriuretic Peptide Precursor A.

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. Al...

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ژورنال

عنوان ژورنال: Journal of Arrhythmia

سال: 2011

ISSN: 1880-4276,1883-2148

DOI: 10.4020/jhrs.27.pj3_057