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Clinical Spectrum of Supravalvular Aortic Stenosis

Arch Intern Med. 1966;118(6):553-561. doi:10.1001/archinte.1966.00290180029006.
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SUPRAVALVULAR aortic stenosis is an unusual congenital entity with diverse clinical manifestations. An intense interest in this subject was initiated by the unexpected finding of this lesion at the necropsy examination of a young man who had been thought to have primary myocardial disease. Impressed by the fact that this operable lesion had not been suspected clinically, we reviewed three other examples of this anomaly in order to present four patients displaying the clinical spectrum of the disease.

Report of Cases 

Case 1.  —A 16-year-old white boy of high intelligence was admitted to The University of Michigan Medical Center in November 1962. He had been well, except for a recent infection of the upper respiratory tract, until five weeks prior to admission when he developed fatigue, dyspnea on exertion, nocturnal cough, orthopnea, paroxysmal nocturnal dyspnea, pleuritic pain of the anterior part of the chest, and hemoptysis. He was hospitalized for


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