Abstract
A 13 yr old boy with Kearns Sayre syndrome, consisting of retinitis pigmentosa, external ophthalmoplegia, and myocardial fibrosis, is presented. A review of the clinical findings and electrocardiographic changes over a period of three years showed progressive atrioventricular and intraventricular conduction system disturbances. Electrophysiologic documentation by His bundle recordings localized the atrioventricular (AV) block distal to the AV node. Postmortem examination revealed microscopic degeneration and fibrosis in the distal portion of the bundle of His and the right and left bundle branches. An approach to cardiac evaluation and therapy is discussed.
| Original language | English |
|---|---|
| Pages (from-to) | 727-730 |
| Number of pages | 4 |
| Journal | Chest |
| Volume | 68 |
| Issue number | 5 |
| State | Published - Dec 1 1975 |
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ASJC Scopus subject areas
- Pulmonary and Respiratory Medicine
Cite this
Heart block in Kearns Sayre syndrome : electrophysiologic pathologic correlation. / Clark, D. S.; Myerburg, Robert J; Morales, Azorides R.
In: Chest, Vol. 68, No. 5, 01.12.1975, p. 727-730.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Heart block in Kearns Sayre syndrome
T2 - electrophysiologic pathologic correlation
AU - Clark, D. S.
AU - Myerburg, Robert J
AU - Morales, Azorides R
PY - 1975/12/1
Y1 - 1975/12/1
N2 - A 13 yr old boy with Kearns Sayre syndrome, consisting of retinitis pigmentosa, external ophthalmoplegia, and myocardial fibrosis, is presented. A review of the clinical findings and electrocardiographic changes over a period of three years showed progressive atrioventricular and intraventricular conduction system disturbances. Electrophysiologic documentation by His bundle recordings localized the atrioventricular (AV) block distal to the AV node. Postmortem examination revealed microscopic degeneration and fibrosis in the distal portion of the bundle of His and the right and left bundle branches. An approach to cardiac evaluation and therapy is discussed.
AB - A 13 yr old boy with Kearns Sayre syndrome, consisting of retinitis pigmentosa, external ophthalmoplegia, and myocardial fibrosis, is presented. A review of the clinical findings and electrocardiographic changes over a period of three years showed progressive atrioventricular and intraventricular conduction system disturbances. Electrophysiologic documentation by His bundle recordings localized the atrioventricular (AV) block distal to the AV node. Postmortem examination revealed microscopic degeneration and fibrosis in the distal portion of the bundle of His and the right and left bundle branches. An approach to cardiac evaluation and therapy is discussed.
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UR - http://www.scopus.com/inward/citedby.url?scp=0016764208&partnerID=8YFLogxK
M3 - Article
AN - SCOPUS:0016764208
VL - 68
SP - 727
EP - 730
JO - Chest
JF - Chest
SN - 0012-3692
IS - 5
ER -