Women with complex congenital heart disease, such as those with single-ventricle physiology, are surviving into adulthood and becoming pregnant. Because of their complex physiology, common peripartum complications pose unique risks. We describe a patient with a single ventricle who underwent an external vascular conduit, nonfenestrated Fontan procedure in childhood and then presented during the postpartum period with extensive thrombosis in her lower extremity deep venous system and inferior vena cava. In this article, we will discuss single-ventricle physiology and the implications of pregnancy, anesthetic considerations, and data for maternal and fetal outcomes in this population.
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