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.
MA, S., MS, Y., MA, Z., & K, S. (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.. BMJ case reports. https://doi.org/10.1136/bcr-2025-271192
MA S, MS Y, MA Z, K S. 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
MA S, MS Y, MA Z, et al. 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.[J]. BMJ case reports. 2026. DOI: 10.1136/bcr-2025-271192.
@article{ma2026,
author = {Sharafat MA and Yousuf MS and Zahid MA and Samad K},
title = {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.},
journal = {BMJ case reports},
year = {2026},
doi = {10.1136/bcr-2025-271192},
note = {PMID: 42442851},
}
TY - JOUR AU - Sharafat MA AU - Yousuf MS AU - Zahid MA AU - Samad K TI - 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. T2 - BMJ case reports PY - 2026 DO - 10.1136/bcr-2025-271192 AN - PMID:42442851 ER -
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.