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Sinus dysrhythmia in Kearns-Sayre syndrome
K S Ulicny1, F C Detterbeck, C D Hall
1Department of Surgery, University of North Carolina School of Medicine, Chapel Hill.
Pacing and Clinical Electrophysiology : PACE
|May 1, 1994
Summary
Kearns-Sayre syndrome, a rare disorder, can cause sinus node dysfunction, including prolonged pauses. This case highlights previously unreported electrocardiographic findings in affected patients.
Area of Science:
- Cardiology
- Genetics
- Neurology
Background:
- Kearns-Sayre syndrome (KSS) is characterized by progressive external ophthalmoplegia, pigmentary retinopathy, and atrioventricular block.
- The underlying etiology is often linked to mitochondrial DNA deletions.
- Previous electrocardiographic findings in KSS typically include various forms of AV block and conduction abnormalities.
Observation:
- This report details a case of Kearns-Sayre syndrome in an asymptomatic patient.
- The patient exhibited previously undocumented episodes of sinus arrest.
- These sinus arrest events resulted in significant cardiac pauses, lasting up to 6 seconds.
Findings:
- Sinus node dysfunction, specifically sinus arrest, is a potential manifestation of Kearns-Sayre syndrome.
- The observed cardiac pauses were prolonged, indicating significant sinoatrial node impairment.
- This finding expands the spectrum of known electrocardiographic abnormalities associated with KSS.
Implications:
- Recognizing sinus node dysfunction in KSS is crucial for accurate diagnosis and management.
- Prolonged sinus pauses may necessitate cardiac monitoring or intervention.
- This case underscores the importance of comprehensive electrocardiographic evaluation in patients with Kearns-Sayre syndrome.