Clinical implications and possible association of malposition of the branch pulmonary arteries with DiGeorge syndrome and microdeletion of chromosomal region 22q11.
We describe a series of 10 patients with malposition of the branch pulmonary arteries (4 patients with crossing [crossed pulmonary arteries] and 6 patients without crossing), 2 of whom had a short main pulmonary artery segment that resulted in iatrogenic right pulmonary artery stenosis after pulmonary artery band placement. DiGeorge syndrome was seen in 5 patients and 4 had microscopic deletion of chromosomal region 22q11.
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Division of Pediatric Cardiology, Mount Sinai Medical Center, New York, New York, USA.
Parness
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Gelb
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BD
Lopez
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Lai
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WW
D000293:Adolescent; D002872:Chromosome Deletion; D002892:Chromosomes, Human, Pair 22; D004062:DiGeorge Syndrome / Q000150:complications* / Q000235:genetics; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications / Q000235:genetics; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities*