MFM Perspective: Extracardiac Considerations and Perinatal Management

Heterotaxy syndromes (1/10, 000 people) are characteristized by situs ambiguity, complex cardiac abnormalities and extra cardiac abnormalities. Etiologies remain elusive but genetic abnormalities should be considered with amniocentesis offered for karyotype, microarray and even studies enrolling patients for exome and whole exome sequencing. Multidisciplinary management is critical with emphasis placed upon the presence of cardiac abnormalities which will greatly impact overall survival and drive location of delivery. In addition to targeted ultrasound and echocardiography, fetal MRI is helpful to enhance detection of extra cardiac abnormalities.