SUPRAVALVULAR AORTIC AND PERIPHERAL PULMONARY ARTERIAL STENOSES - A REPORT OF 8 CASES IN 2 GENERATIONS

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  • 1 January 1980
    • journal article
    • research article
    • Vol. 16  (7) , 496-502
Abstract
Peripheral pulmonary arterial stenosis, alone or in combination with supravalvular aortic stenosis, is described in 2 generations of a family. The last child born in the 1st generation was the only fatal case and showed severe narrowing and thrombosis of the pulmonary arteries and significant narrowing of the descending aorta. Physical and mental development were normal in the 7 surviving patients. Five had slight dyspnea on effort. Hemodynamic and angiocardiographic studies showed multiple peripheral pulmonary stenosis in 6 patients and supravalvular aortic stenosis or aortic hypoplasia in the last 3 of the 1st generation and in one of the 2nd generation. The younger children were more severely affected. A marked systolodiastolic caliber variation of the main pulmonary arteries was noted angiographically in all those studied. This finding can be used as an indirect sign of the presence of peripheral pulmonary artery stenosis.

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