[Coronary anomalies associated with the Williams-Beuren syndrome. Apropos of 2 cases]

Abstract

Two coronary artery anomalies associated with the Williams-Beuren syndrome are reported. The first case was a 14 year old child with severe supra-aortic stenosis associated with severe hypoplasia of the ascending aorta; 2 D echocardiography and angiography showed a voluminous aneurysm of the left main coronary artery. Aortoplasty with a patch gave a good early result. The second case was a two and a half months old baby operated as an emergency after cardiac arrest and who died at the end of surgery. The baby had severe supra-aortic stenosis, occlusion of the left coronary ostium by the left anterior cusp which was abnormally adherent to the aortic wall with subendocardial infarction and reaction fibroelastosis. The frequency of coronary anomalies associated with the Williams-Beuren syndrome is probably underestimated. Because of their severity they must be looked for routinely, by echocardiography and angiography. Their tendency to progress is an indication for early surgical correction of severe supra-aortic stenosis.