Early onset of progressive subaortic stenosis after complete repair of tetralogy of Fallot

Tetralogy of Fallot is often found in association with a wide variety of ot her cardiac lesions, but is rarely found in association with lesions causin g obstruction to the left ventricular inflow or outflow. Subaortic stenosis has only rarely been reported in association with tetralogy of Fallot. We report a patient with Marfan syndrome who underwent repair of tetralogy of Fallot at five years of age. Discrete and progressive subaortic stenosis de veloped two years after the surgical correction, in a previously normal and unobstructed left ventricular outflow tract. Surgical removal of the acqui red fibrous subaortic shelf was successful. Clinical signs of obstruction w ithin the left ventricular outflow tract after surgical repair of tetralogy of Fallot should prompt further investigation to exclude the onset of acqu ired subaortic stenosis.