Abstract:
:Two sisters who presented with diffuse hypoplasia of pulmonary arteries, relative hypoplasia of ascending aorta, obstructive uropathy, bilateral ureteral reflux, and hydronephrosis, are described. The subsequent course was characterized by progressive and gradual onset of right heart failure, failure to thrive, chronic malabsorption and systemic hypertension. The syndrome which appears to be transmitted by autosomal recessive inheritance can possibly represent a generalized hypoplasia and growth failure of a part or the entire arterial system. Peripheral pulmonary stenosis can occur as an isolated lesion or in association with other congenital cardiac anomalies, as well as in rubella syndrome and the syndrome of supravalvular aortic stenosis. This communication reports two siblings with a hitherto unreported combination of hypoplastic pulmonary arteries, and aorta with identical genito-urinary tract abnormalities.
journal_name
Angiologyjournal_title
Angiologyauthors
Kashani IA,Strom CM,Utley JE,Marin-Garcia J,Higgins CBdoi
10.1177/000331978403500408subject
Has Abstractpub_date
1984-04-01 00:00:00pages
252-6issue
4eissn
0003-3197issn
1940-1574journal_volume
35pub_type
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