The progress of 128 patients with congenital aortic stenosis has been followed from one to 28 (mean 14) years. Fifty-eight underwent cardiac catheterisation, and 46 (36% of the total) required surgical treatment. Of these, 42 were under 20 years old. Additional cardiac lesions were noted in five. Infective endocarditis was encountered in four. The onset of symptoms or increasing evidence of left ventricular hypertrophy on the electrocardiogram were the principal indications for catheterisation. Two-dimensional echocardiography is now important in this context. There were four deaths in the 46 surgically treated patients; three of these were early and the fourth was a late death three years after operation due to a massive cerebral embolus complicating infective endocarditis. The 42 survivors of operation and the 82 unoperated patients have remained under long-term supervision. Further surgery was necessary in 12 of the 42 surgically treated patients--valve replacement in seven of them two to eight years after valvotomy, replacement of a calcified xenograft valve in three, and repeat operation in two because of recurrence of subvalvar obstruction. Aortic stenosis is not a benign condition in childhood and adolescence. Close supervision is necessary and when any deterioration is detected further investigation as a prelude to probable surgery is mandatory. This should not be embarked on lightly in childhood unless there are pressing indications, particularly in view of the serious disadvantages of valve replacement in childhood.
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