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Primary oxalosis with pan-conduction cardiac disease: electrophysiologic and anatomic correlation
Insights
Primary oxalosis can cause severe cardiac issues. This case highlights oxalate infiltration of the heart
Area of Science:
- Nephrology
- Cardiology
- Electrophysiology
Background:
- Primary oxalosis is a rare metabolic disorder leading to systemic oxalate deposition.
- Chronic hemodialysis is a common treatment for end-stage renal disease in such patients.
- Extensive visceral involvement is a known complication of primary oxalosis.
Observation:
- A 27-year-old male with primary oxalosis on hemodialysis presented with presyncope.
- Electrocardiogram revealed erratic atrial rhythm, atrioventricular dissociation, and right bundle branch block.
- Electrophysiologic studies demonstrated complex atrial and conduction abnormalities.
Findings:
- Irregular atrial depolarizations and inexcitability were observed.
- Atrioventricular dissociation and infranodal conduction delay were significant findings.
- Extensive oxalate infiltration was identified in cardiac conduction tissues, including the sinoatrial node, AV node, and His bundle.
Implications:
- This is the first report of cardiac electrophysiologic abnormalities directly linked to oxalate infiltration in primary oxalosis.
- Oxalate deposition can significantly disrupt cardiac electrical activity.
- Understanding these mechanisms is crucial for managing cardiac complications in primary oxalosis.
Abstract:
A 27-year-old man with primary oxalosis and extensive visceral involvement was maintained on long-term chronic hemodialysis. He had an episode of presyncope associated with electrocardiographic findings of an erratic atrial rhythm, atrioventricular dissociation due to an accelerated junctional rhythm and right bundle branch block. Electrophysiologic studies showed irregular atrial depolarizations probably associated with multiple atrial pacemakers, atrial inexcitability, atrioventricular dissociation and infranodal conduction delay. These findings correlated well with extensive oxalate infiltration of the sinoatrial node and its approaches, the atrial preferential pathways, the approaches to the atrioventricular node, the atrioventricular node, and the His bundle and bundle branches. This is the first reported case of cardiac electrophysiologic abnormalities due to oxalate infiltration in a patient with primary oxalosis.