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Supravalvular aortic and peripheral pulmonary arterial stenoses. A report of eight cases in two generations.
- Source :
-
Israel journal of medical sciences [Isr J Med Sci] 1980 Jul; Vol. 16 (7), pp. 496-502. - Publication Year :
- 1980
-
Abstract
- Peripheral pulmonary arterial stenosis, either alone or in combination with supravalvular aortic stenosis, is described in two generations of one family. The last child born in the first generation ws 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 seven surviving patients. Five had slight dyspnea on effort. Hemodynamic and angiocardiogrphic studies showed multiple peripheral pulmonary stenosis in six patients and supravalvular aortic stenosis or aortic hypoplasia in the last three of the first generation and in one of the second 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. We suggest that this finding can be used as an indirect sign of the presence of peripheral pulmonary arterial stenosis.
- Subjects :
- Aortic Valve Stenosis complications
Aortography
Blood Pressure
Electrocardiography
Female
Follow-Up Studies
Heart Atria pathology
Heart Ventricles pathology
Humans
Male
Pedigree
Pulmonary Artery diagnostic imaging
Pulmonary Valve Stenosis complications
Radiography, Thoracic
Aortic Valve Stenosis genetics
Pulmonary Valve Stenosis genetics
Subjects
Details
- Language :
- English
- ISSN :
- 0021-2180
- Volume :
- 16
- Issue :
- 7
- Database :
- MEDLINE
- Journal :
- Israel journal of medical sciences
- Publication Type :
- Academic Journal
- Accession number :
- 7399883