A Novel Association of Biventricular Cardiac Noncompaction and Diabetic Embryopathy: Case Report and Review of the Literature

Diabetic embryopathy refers to a constellation of congenital malformations arising in the setting of poorly controlled maternal diabetes mellitus. Cardiac abnormalities are the most frequently observed findings, with a 5-fold risk over normal pregnancies. Although a diverse spectrum of cardiac defec...

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Bibliographic Details
Published inPediatric and developmental pathology Vol. 18; no. 1; pp. 71 - 75
Main Authors Woo, Jennifer S., Perez-Rosendahl, Mari, Haydel, Dana, Perens, Gregory, Fishbein, Michael C.
Format Journal Article
LanguageEnglish
Published Los Angeles, CA SAGE Publications 01.01.2015
SAGE PUBLICATIONS, INC
Subjects
Abnormalities, Multiple - diagnosis
Diabetes Complications
Echocardiography - methods
Fatal Outcome
Female
Fetal Diseases - pathology
Heart - embryology
Heart Defects, Congenital
Heart Diseases - congenital
Heart Diseases - diagnosis
Heart Ventricles - embryology
Heart Ventricles - pathology
Humans
Myocardium - pathology
Pregnancy
Pregnancy Complications
Pregnancy in Diabetics
congenital heart disease
diabetic embryopathy
cardiac noncompaction
embryogenesis
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Summary:Diabetic embryopathy refers to a constellation of congenital malformations arising in the setting of poorly controlled maternal diabetes mellitus. Cardiac abnormalities are the most frequently observed findings, with a 5-fold risk over normal pregnancies. Although a diverse spectrum of cardiac defects has been documented, cardiac noncompaction morphology has not been associated with this syndrome. In this report, we describe a novel case of biventricular cardiac noncompaction in a neonate of a diabetic mother. The patient was a late preterm female with right anotia, caudal dysgenesis, multiple cardiac septal and aortic arch defects, and biventricular cardiac noncompaction. Examination of both ventricles demonstrated spongy myocardium with increased myocardial trabeculation greater than 50% left ventricular thickness and greater than 75% right ventricular thickness, with hypoplasia of the bilateral papillary muscles, consistent with noncompaction morphology. Review of the literature highlights the importance of gene expression and epigenomic regulation in cardiac embryogenesis.
Bibliography:ObjectType-Case Study-3
SourceType-Scholarly Journals-1
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ObjectType-Review-1
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ISSN:1093-5266
1615-5742
DOI:10.2350/14-07-1532-CR.1