We report a case of 24-year-old man with single ventricle congenital heart disease and concomitant familial Fabry disease who developed progressive subaortic stenosis 22 years after Fontan procedure. Despite stable ventricular volumes, subaortic stenosis developed due to Fabry disease-related myocardial hypertrophy. He underwent pulmonary arterial trunk reconstruction with valved conduit and Damus–Kaye–Stansel (DKS) anastomosis. Postoperatively, smooth outflow tracts and stable valve function were observed. This case highlights the impact of Fabry disease on ventricular outflow tract obstruction and shows the efficacy of the DKS anastomosis in treating subaortic stenosis.
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