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[Pacemaker therapy in Kearns-Sayre syndrome]
1Department of Thoracic Surgery, Nagoya University School of Medicine, Nagoya, Japan.
Kyobu Geka. the Japanese Journal of Thoracic Surgery
|December 13, 2002
Summary
Kearns-Sayre syndrome, a mitochondrial disorder, can cause severe heart block. Pacemaker implantation is a crucial treatment for affected individuals, especially those with bifascicular block, improving cardiac function.
Area of Science:
- Neurology
- Cardiology
- Genetics
Background:
- Kearns-Sayre syndrome (KSS) is a rare mitochondrial myopathy characterized by specific clinical triad.
- It involves mitochondrial DNA (mtDNA) deletions, leading to multi-systemic effects.
- Diagnosis relies on progressive external ophthalmoplegia, pigmentary retinopathy, and cardiac conduction defects.
Observation:
- This report details two KSS cases requiring pacemaker implantation.
- Patient 1, a 20-year-old male, developed complete heart block from bifascicular block.
- Patient 2, a 27-year-old female, experienced torsade de pointes with complete heart block.
Findings:
- Pacemaker implantation effectively managed cardiac conduction abnormalities in both KSS patients.
- Bifascicular block in KSS is a strong indicator for prophylactic pacemaker implantation.
- Challenges in KSS pacing include patient size, mode selection, and congenital heart disease.
Implications:
- Early electrocardiography screening is vital for KSS patients.
- Pacemaker implantation is a recommended intervention for KSS patients with conduction blocks.
- Understanding pacing challenges can optimize management for KSS patients.