Progression of Conduction System Disease in a Paced Patient With Kearns‐Sayre Syndrome

Abstract

The Kearns‐Sayre syndrome is a rare condition characterized by external ophthalmoplegia, retinal pigmentary degeneration and progressive impairment of cardiac conduction. We report a patient with the permanent pacemaker. The spontaneous cardiac impulse formation disappeared during long‐term follow‐up in this patient with Kearns‐Sayre Syndrome. Copyright © 2009 Wiley Periodicals, Inc.

2022 HRS expert consensus statement on evaluation and management of arrhythmic risk in neuromuscular disorders

“…In chronic progressive external ophthalmoplegia, most commonly in the Kearns-Sayre syndrome variant, cardiac involvement manifests primarily as conduction abnormalities including sinus node dysfunction and progressive AV block. 184,[197][198][199][201][202][203] In Kearns-Sayre syndrome, AV block is observed usually after the onset of eye involvement. The HV interval is prolonged, consistent with distal conduction disease.…”

“…Those with mitochondrial myopathies are much more susceptible to developing symptomatic AV (particularly infranodal) conduction disturbances rather than sinoatrial node dysfunction, with the former generally being considered more severe and associated with progression. 184,197,199,203 Discovery of significant bradycardia following the onset of mild or transient symptoms, particularly that due to AV block, may therefore provide a critical opportunity to offer early pacemaker implantation to a patient with mitochondrial myopathy, possibly avoiding the occurrence of more serious symptoms and even death from evolution to advanced persistent AV block. 2.…”

“…Pacemaker implantation may not only lead to symptomatic improvement but may provide prognostic benefit. 184,[197][198][199][201][202][203] 3. CRT has not been specifically studied in mitochondrial myopathies or FA, although evidence derived from the CRT literature has been extrapolated to these conditions with appropriate cardiovascular substrate, clinical features, and guideline-directed medical therapy.…”

“…The severity of conduction disease at which pacing should be instituted is not clear but should reflect a significant burden or progression of impairment. 184,[197][198][199][201][202][203] 7.4. Atrial arrhythmias in mitochondrial myopathies including Friedreich ataxia Synopsis AF has been observed in patients with mitochondrial disorders primarily in chronic progressive external ophthalmoplegias and FA, and its presence may signify progression of underlying structural cardiac involvement.…”