Excerpt

A 23yo G4P3 woman presented at 20 weeks gestation to our institution with concern for complex congenital heart disease in the fetus. Fetal echocardiography (Figs. 1 and 2) was consistent with double outlet right ventricle with subaortic obstruction and aortic coarctation, as well as superior inferior relationships of the ventricles. The fetus delivered at 38 weeks gestation. Initial transthoracic imaging confirmed the diagnosis but was unable to clearly represent the three-dimensional relationships of the ventricles to each other and of the heart to sternum as clearly as fetal imaging. An MRI was ordered which confirmed the prenatal diagnosis and aided in surgical planning of the Stage I procedure. The MRI (Figs. 3 and 4) identified the presence of an infundibulum, and the fact that the outflow tracts were leftward, and the infant underwent Norwood/Sano procedure. Identification of the infundibulum preoperatively aided in determining that the Sano modification would be possible. The baby has since had a bidirectional Glenn procedure and is doing well. At time of surgery, the heart was noted to be mesocardic, with no clear apex, and confirmed the unusual position of the hypoplastic left ventricle superior and in a “U shaped” orientation with the septum horizontal. Fetal cardiac imaging can detail cardiac segmental anatomy and cardiac chamber structure and position accurately even for complex anatomy and may surpass postnatal echo in some instances for assessing cardiac position given that the lungs are not an acoustic barrier. The combination of fetal echocardiography, postnatal echocardiography, and cardiac MRI allowed for successful surgical planning.

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