Abstract
Left main coronary artery (LMCA) stenosis accompanying to supravalvular aortic stenosis is a very uncommon, serious congenital abnormality. Aortic valve leaflet fusions and intimal thickening of the aortic valve leaflets and coronary artery are the underlying pathologies for the LMCA stenosis. We operated on a 21-year-old male patient for supravalvar aortic stenosis with LMCA ostial stenosis. We enlarged the LMCA with a pericardial patch (ostial plasty) and reconstructed the aortic root with a modified Brom procedure. Postoperative course was uneventful; echocardiographic evaluation revealed a normal functioning aortic valve with a normal left ventricular function. Gradient at left ventricular outflow tract was decreased a great deal. Although supravalvular aortic stenosis with LMCA stenosis is a very rare congenital abnormality, this clinical entity can be successfully corrected with detailed and selected surgical procedures.
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