Objective:

To describe a patient with Ehlers-Danlos syndrome (EDS) suffering very rare neurologic complications after developing an atrial-esophageal fistula (AEF) as a complication of catheter ablation for atrial fibrillation. 

Background:

Catheter ablation treatment in atrial fibrillation carries a rare but serious risk of  AEF development (<0.1% of patients). Symptoms of AEF such as fever (73%), neurological complications (72%), and gastrointestinal issues (40%) are delayed and can be life-threatening (55% mortality). The understanding of the safety of cardiac procedures for individuals with EDS and its neurological complications remains limited.

Design/Methods:

NA

Results:

A 48-year-old male with a history of EDS, atrial fibrillation, and benzodiazepine overuse experienced severe symptoms after an AV nodal ablation. Initially, he developed fever, headache, left homonymous hemianopia, right gaze preference, and left hemiplegia. Brain imaging revealed extensive frontal cortical hemorrhage, multivascular acute ischemic infarcts, and imaging findings indicative of an abscess and septic emboli. Blood cultures indicated the presence of alpha-hemolytic streptococcus, Prevotella species, and Candida glabrata, persisting despite antimicrobial treatment. Craniotomy for abscess drainage disclosed the presence of Enterococcus faecalis and Lactobacillus species. Serial echocardiograms did not show vegetations but chest CT identified an AEF requiring surgical repair. After a prolonged hospital stay, the patient was discharged to acute rehabilitation, demonstrating significant improvement and enhanced independence in daily activities.

Conclusions:

Early recognition of AEF symptoms following cardiac procedures in patients with EDS is crucial to avoid serious complications. Underlying cardiac tissue fragility in EDS may predispose patients to this condition.  To our knowledge, this is the only reported case of simultaneous ischemic, hemorrhagic and infectious complications of AEF.