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DOI 10.1136/bcr-2025-271192

Медицинская библиотека · BMJ case reports · 2026

Anaesthetic management of a child with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) and supravalvular aortic stenosis (SVAS) associated with Williams-Beuren syndrome undergoing combined coronary reimplantation and supravalvular aortoplasty.

Sharafat Muhammad Azhar, Yousuf Muhammad Saad, Zahid Muhammad Arslan, Samad Khalid

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Аннотация

A child with genetically confirmed Williams-Beuren syndrome, having moderate supravalvular aortic stenosis, was diagnosed with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), a rarely described combination. This coexistence created competing physiological stress on coronary perfusion as myocardial oxygen delivery to the left ventricle depended on right coronary collateral circulation in the presence of fixed left ventricular outflow obstruction. The child underwent combined surgical repair with reimplantation of the left coronary artery into the aorta and supravalvular aortoplasty. Anaesthesia was induced gradually to avoid abrupt haemodynamic changes, invasive monitoring was established early, and ventilation was adjusted to maintain normocapnia and moderate oxygenation to limit factors that could aggravate coronary steal physiology. The child was separated from bypass on minimal inotropic support and had an uneventful postoperative recovery. This case illustrates the relevance of controlled induction, meticulous haemodynamic balance and coordinated team management for a successful outcome in such a complex case.

Для цитирования: Sharafat Muhammad Azhar, Yousuf Muhammad Saad, Zahid Muhammad Arslan, Samad Khalid. Anaesthetic management of a child with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) and supravalvular aortic stenosis (SVAS) associated with Williams-Beuren syndrome undergoing combined coronary reimplantation and supravalvular aortoplasty.. BMJ case reports. 2026. DOI: 10.1136/bcr-2025-271192.

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