Key result
Transthoracic echocardiography guided the perioperative management of a patient with Williams syndrome, aortic arch pseudoaneurysm, and infective vegetation undergoing a modified Broms procedure.
Why the study?
The association of infective endocarditis within the aortic arch and pseudoaneurysm formation in Williams-Beuren syndrome is infrequent, and perioperative management is challenging.
Case Report (n=1)
Highlights the rare association of infective endocarditis and pseudoaneurysm in Williams-Beuren syndrome and the utility of transthoracic echocardiography in perioperative management.
Supports individualized TTE use in rare Williams syndrome cases; leaves open broader validation.
Williams-Beuren syndrome is a rare genetic malformation with predilection for supravalvular aortic stenosis. Apart from cardiovascular malformation, hypocalcemia, developmental delay, and elfin facies, challenging airway make perioperative management more eventful. Association of infective endocarditis within the aortic arch and pseudoaneurysm formation is infrequent. We, hereby report a case of pseudoaneurysm formation and infective vegetation within the aortic arch in a patient with Williams syndrome and the role of transthoracic echocardiography in its perioperative management.
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Das et al. (2023) conducted a case report in Williams-Beuren syndrome with aortic arch pseudoaneurysm and infective vegetation (n=1). Transthoracic echocardiography and modified Broms procedure was evaluated. Transthoracic echocardiography guided the perioperative management of a patient with Williams syndrome, aortic arch pseudoaneurysm, and infective vegetation undergoing a modified Broms procedure.
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