TITLE

Hypoplastic Pulmonary Arteries and Aorta with Obstructive Uropathy in 2 Siblings

AUTHOR(S)
Kashani, Iraj A.; Strom, Charles M.; Utley, Joseph E.; Marin-Garcia, Jose; Higgins, Charles B.
PUB. DATE
April 1984
SOURCE
Angiology;Apr1984, Vol. 35 Issue 4, p252
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
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 progres- sive 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 lesion1�7 or in association with other congenital cardiac anomalies, as well as in rubella syndrome8,9 and the syndrome of supravalvular aortic stenosis.10,11 This communication reports two siblings with a hitherto unreported combination of hypoplastic pulmonary arteries, and aorta with identical genito-urinary tract abnormalities.
ACCESSION #
16352503

 

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