Subaortic stenosis after surgical correction of a partial atrioventricular septal defect is uncommon. When it occurs, it is usually diagnosed 6-8 years after early surgical repair in childhood. We present a female patient who underwent mitral valve cleft repair and closure of a partial atrial septal defect with a Teflon patch at the age of 48 years. Twenty-two years later, she presented with severe subaortic stenosis that required surgical repair. We analyze the intracardiac morphological factors associated with the development of late subaortic stenosis. We recommend long-term follow-up, even after surgical repair in adults.

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