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A case of supravalvular pulmonary stenosis associated with Noonan's syndrome: usefulness of Doty's operation]
Authors:T Sugiura  Y Takahara  Y Sudo  H Murayama  I Nakada  T Sezaki  T Nakamura
Affiliation:Department of Cardiovascular Surgery, Tsurumai Hospital.
Abstract:A twelve-year-old boy associated with Noonan's syndrome is reported. He had supravalvular pulmonary stenosis with a very high right-ventricular pressure, higher than that of systemic. Surgical treatment was performed to the supravalvular pulmonary stenosis with the technique of Doty's operation for supravalvular aortic stenosis, brought an excellent results. The gradient between the right ventricle and the pulmonary artery fell from 97 mmHg to 30 mmHg. The residual gradient is caused by functional infundibular stenosis. Doty's operation is a useful method for the operation of severe supravalvular pulmonary stenosis, too.
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