Williams-Beuren syndrome with pseudoaneurysm of aortic arch and infective vegetations for modified broms procedure: anesthetic concerns & Echocardiographic illustrations

Devishree Das; Neeti Makhija

doi:10.4103/aca.aca_122_21

Williams-Beuren syndrome with pseudoaneurysm of aortic arch and infective vegetations for modified broms procedure: anesthetic concerns & Echocardiographic illustrations

Ann Card Anaesth. 2023 Jan-Mar;26(1):94-96. doi: 10.4103/aca.aca_122_21.

Authors

Devishree Das¹, Neeti Makhija¹

Affiliation

¹ Department of Cardiac Anaesthesia, Cardiothoracic Centre, CNC, All India Institute of Medical Sciences, New Delhi, Ansari Nagar, New Delhi, India.

Abstract

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.

Keywords: Echocardiography; infective endocarditis; modified broms procedure; pseudoaneurysm of aortic arch; supravalvular aortic stenosis; williams-beuren syndrome.

Publication types

Case Reports

MeSH terms

Anesthetics*
Aneurysm, False* / diagnostic imaging
Aneurysm, False* / surgery
Aorta, Thoracic / diagnostic imaging
Aorta, Thoracic / surgery
Echocardiography
Humans
Williams Syndrome* / complications
Williams Syndrome* / diagnostic imaging

Substances

Anesthetics