Paradoxical Cerebral Air Embolism after Cardiac Ablation in Williams-Beuren Syndrome: A Clinico-Pathological Correlation

Non-traumatic neurological deterioration is a medical emergency that may arise from diverse causes, to include cerebral infarction or intracranial hemorrhage, meningoencephalitis, seizure, hypoxic-ischemic or toxic/metabolic encephalopathy, poisoning, or drug intoxication. We describe the abrupt ons...

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Published in:Journal of stroke and cerebrovascular diseases 2022-04, Vol.31 (4), p.106317-106317, Article 106317
Main Authors: Ionel, Dana L., Odago, Fred O., Ene, Andre N., Lee, Jessica D., O'Connor, William N., Goldstein, Larry B., Pettigrew, L. Creed
Format: Article
Language:English
Subjects:
Aortic Stenosis, Supravalvular - genetics
Aortic Stenosis, Supravalvular - pathology
Atrial fibrillation
Atrial Fibrillation - complications
Atrio-esophageal fistula
Catheter Ablation - adverse effects
Cerebral air embolism
Embolism, Air
Humans
Pulmonary vein isolation
Williams Syndrome - complications
Williams Syndrome - diagnosis
Williams Syndrome - genetics
Williams-Beuren syndrome
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Summary:Non-traumatic neurological deterioration is a medical emergency that may arise from diverse causes, to include cerebral infarction or intracranial hemorrhage, meningoencephalitis, seizure, hypoxic-ischemic or toxic/metabolic encephalopathy, poisoning, or drug intoxication. We describe the abrupt onset of neurological deterioration in a 53-year-old man with Williams-Beuren syndrome, a sporadically occurring genetic disorder caused by chromosomal microdeletion at 7q11.23. The clinical phenotype of Williams-Beuren syndrome is suggested by distinctive elfin facies, limited intellect, unique personality features, growth abnormalities, and endocrinopathies. The causative microdeletion of chromosomal material will frequently involve loss of the elastin gene, ELN, with resulting arteriopathy, supravalvular aortic stenosis, non-ischemic cardiopathy, and atrial fibrillation. Our patient sustained acute neurological decline within one month after undergoing a cardiac ablative procedure to convert atrial fibrillation to sinus rhythm. We present our findings in the setting of a clinico-pathological correlation, in which we reveal the cause of the abrupt neurological deterioration and discuss how our patient was affected by an uncommon stroke disorder.
ISSN:1052-3057
1532-8511
DOI:10.1016/j.jstrokecerebrovasdis.2022.106317