Noonan syndrome with atrial septal defect and hypertrophic cardiomyopathy

: Noonan syndrome is one of the most common nonchromosomal syndromes seen in children with congenital heart disease. The major cause of death is congestive heart failure. We report a case of Noonan syndrome with hypertrophic cardiomyopathy and arterial septal defect (ASD). Since 10 years old he had been suffering from congestive heart failure. ASD patch closure with a fenestrated flap valve patch was performed at 12 years of age. After operation, left-to-right shunt flow through the patch gradually decreased and he was discharged at 19 postoperative day in good condition.