Dextrocardia with and without situs viscerum inversus in two sibs

We described two sibs born to consanguineous Sicilian parents who died of severe congenital heart malformation. Both had dextrocardia; however, only the girl had situs viscerum inversus. At necropsy she was found to have a right spleen and right pulmonary isomerism (three lobes in each lung, as comm...

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Bibliographic Details
Published in:	American journal of medical genetics Vol. 27; no. 4; p. 929
Main Authors:	Distefano, G, Romeo, M G, Grasso, S, Mazzone, D, Sciacca, P, Mollica, F
Format:	Journal Article
Language:	English
Published:	United States 01-08-1987
Subjects:	Abnormalities, Multiple - diagnostic imaging Consanguinity Dextrocardia - diagnostic imaging Female Genes, Recessive Heart Defects, Congenital - diagnostic imaging Humans Infant, Newborn Lung - abnormalities Lung - diagnostic imaging Male Radiography Spleen - abnormalities Spleen - diagnostic imaging Syndrome Transposition of Great Vessels - diagnostic imaging Viscera - abnormalities Viscera - diagnostic imaging
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Summary:	We described two sibs born to consanguineous Sicilian parents who died of severe congenital heart malformation. Both had dextrocardia; however, only the girl had situs viscerum inversus. At necropsy she was found to have a right spleen and right pulmonary isomerism (three lobes in each lung, as commonly found in the asplenia syndrome). This observation, together with other literature reports, suggest that isolated dextrocardia, situs viscerum inversus, and the asplenia-polysplenia complex may be different end results of a unique dysmorphogenetic process involving the embryonic midline.
ISSN:	0148-7299
DOI:	10.1002/ajmg.1320270420