Complete heart block in the 18p--syndrome. Congenital calcification of the atrioventricular node.
Complete heart block exists when the atria and ventricles beat completely independently of each other. It is heterogeneous with respect to pathogenesis. Occasionally, complete heart block may be symptomatic in infancy. Rarely, it is associated with genetic syndromes. Cardiac abnormalities are unusual in the 18p-syndrome. We describe a female stillborn infant who had 18p-syndrome with hydrops fetalis and complete heart block secondary to atrioventricular node calcification.
Bridge
J A
JA
Department of Pathology and Oncology, University of Kansas Medical Center, Kansas City.
McManus
B M
BM
Remmenga
J
J
Cuppage
F P
FP
D000015:Abnormalities, Multiple / Q000150:complications* / Q000473:pathology; D001283:Atrioventricular Node / Q000473:pathology*; D001344:Autopsy; D002114:Calcinosis / Q000150:complications* / Q000151:congenital / Q000473:pathology; D002872:Chromosome Deletion; D002887:Chromosomes, Human, Pair 18; D005260:Female; D005313:Fetal Death; D006327:Heart Block / Q000209:etiology / Q000473:pathology*; D006329:Heart Conduction System / Q000473:pathology*; D006330:Heart Defects, Congenital / Q000150:complications* / Q000473:pathology; D006801:Humans; D015160:Hydrops Fetalis / Q000209:etiology / Q000473:pathology; D007231:Infant, Newborn; D011247:Pregnancy; D013577:Syndrome