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March 1970

Cardiac Malformations With Facial Clefts: With Observations on the Pierre Robin Syndrome

Author Affiliations

From the Department of Medicine, Section of Cardiology (Drs. Shah and Harris), and the Cleft Palate Clinic, Center for Craniofacial Anomalies (Dr. Pruzansky), University of Illinois at the Medical Center, Chicago.

Am J Dis Child. 1970;119(3):238-244. doi:10.1001/archpedi.1970.02100050240010

Among 191 patients with facial clefts (cleft lip and/or cleft palate), the incidence of congenital heart defects was increased. Two-thirds (21 of 32) of the dead children had severe heart disease. In 150 living patients with facial clefts, the incidence of congenital cardiac malformations was 1.3%. In the 31 patients concordant for facial clefts and heart disease, transposition of the great vessels and single ventricle were unexpectedly absent, probably because the embryological defect responsible for these two cardiac lesions precedes palatal development by 1½ to 2 weeks. The incidence of heart disease, while high (20%) in children dying with the Pierre Robin syndrome, was greater (75%) in deceased infants who had cleft palate without the Pierre Robin syndrome.

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