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Cardiac involvement in Emery-Dreifuss muscular dystrophy: role of a diagnostic pacemakerRakovec, Peter ...Emery-Dreifuss muscular dystrophy is an X-linked recessive myopathy. Its progression is slow, and it rarely leads to cessation of walking; therefore, it has often been called "benign." On the other ... hand, cardiac involvement is often severe and sudden death is not uncommon. We describe a family with four affected males, two of whom died suddenly. The case of an affected man with first-degree AV block, incomplete right bundle branch block, and left anterior fascicular block is described in detail. The prophylactic insertion of a diagnostic pacemaker enabled us to follow the progress of conduction disturbances without leaving the patient unprotected. While AV and intraventricular conduction defects were more prominent before pacemaker implantation, sinus node dysfunction became more important during the follow-up.Source: PACE. Pacing and clinical electrophysiology. - ISSN 0147-8389 (Let. 18, št. 9 Pt 1, 1995, str. 1721-1724)Type of material - article, component partPublish date - 1995Language - englishCOBISS.SI-ID - 4681433
Author
Rakovec, Peter |
Zidar, Janez |
Šinkovec, Matjaž, 1955- |
Zupan, Igor, 1961- |
Brecelj, Aleš, 1946-
Topics
Heart block |
Diagnosis |
Etiology |
Muscular dystrophy |
Complications |
Pacemaker, artificial |
Adult |
Atrioventricular node |
Physiopathology |
Bundle-branch block |
Diagnosis |
Etiology |
Death, sudden |
Follow-up studies |
Genes, recessive |
Heart block |
Genetics |
Heart ventricle |
Linkage (genetics) |
Sinoatrial node |
Physiopathology |
X chromosome
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Database name | Field | Year |
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Links to authors' personal bibliographies | Links to information on researchers in the SICRIS system |
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Rakovec, Peter | 09160 |
Zidar, Janez | 08780 |
Šinkovec, Matjaž, 1955- | 06010 |
Zupan, Igor, 1961- | 10726 |
Brecelj, Aleš, 1946- | 02498 |
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