Journal List > Korean J Obstet Gynecol > v.54(10) > 1088351

MD, Kim, Lee, Hwang, Son, and Choi: A CASE OF FETAL HETEROTAXY SYNDROME

Abstract

Heterotaxy syndrome is a disorder that results in abnormal placement of organs. We report the prenatal diagnosis of complex cardiac abnormalities associated with left isomerism. A 32-year-old multigravida was referred to our hospital for evaluation of abnormal sonographic findings. On prenatal sonography, we suspected a double outlet of the right ventricle, right ventricular hypertrophy, a ventricular septal defect, a large azygos vein instead of an inferior vena cava, a right-sided stomach, and asplenia. Postnatal imaging studies confirmed these findings, except a left-sided single spleen. After birth, the infant underwent a left modified Blalock-Taussing shunt and underwent a 2nd surgical procedure (Kawashima operation, right pulmonary artery angioplasty, and atrial septal defect widening). This report suggests intrauterine fetal sonography can accurately delineate abnormal findings in heterotaxy, allowing perinatal counseling and postpartum planning for corrective surgery.

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Fig. 1.
Prenatal sonography showed abnormal findings. A right-sided stomach. st, stomach.
kjog-54-630f1.tif
Fig. 2.
Prenatal sonography showed abnormal findings. Right ventricle larger than left ventricle in a ratio of 1.62 cm:0.63 cm with ventricular septal defect. VSD, ventricular septal defect.
kjog-54-630f2.tif
Fig. 3.
Prenatal sonography showed abnormal findings. Double outlets of right ventricle pulmonary artery [PA]:aorta [AO] = diameter 1.08 cm:0.59 cm).
kjog-54-630f3.tif
Fig. 4.
Prenatal sonography showed abnormal findings. A large azygos vein placed posterior to the aorta. AZY, azygous vein; SVC, superior vena caval.
kjog-54-630f4.tif
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