Korean J Obstet Gynecol.  2011 Oct;54(10):630-633. 10.5468/KJOG.2011.54.10.630.

A case of fetal heterotaxy syndrome

  • 1Department of Obstetrics and Gynecology, Gil Hospital, Graduate School of Medicine, Gachon University of Medicine and Science, Incheon, Korea. ksyob@gilhospital.com
  • 2Department of Pediatrics, Gil Hospital, Graduate School of Medicine, Gachon University of Medicine and Science, Incheon, Korea.
  • 3Department of Cardiothoracic Surgery, Gil Hospital, Graduate School of Medicine, Gachon University of Medicine and Science, Incheon, Korea.


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.


Cavopulmonary shunt; Double outlet right ventricle; Heterotaxy; Isomerism; Prenatal ultrasonography

MeSH Terms

Azygos Vein
Double Outlet Right Ventricle
Heart Bypass, Right
Heart Septal Defects, Atrial
Heart Septal Defects, Ventricular
Heart Ventricles
Heterotaxy Syndrome
Hypertrophy, Right Ventricular
Postpartum Period
Prenatal Diagnosis
Pulmonary Artery
Ultrasonography, Prenatal
Vena Cava, Inferior


  • Fig. 1. Prenatal sonography showed abnormal findings. A right-sided stomach. st, stomach.

  • 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.

  • Fig. 3. Prenatal sonography showed abnormal findings. Double outlets of right ventricle pulmonary artery [PA]:aorta [AO] = diameter 1.08 cm:0.59 cm).

  • Fig. 4. Prenatal sonography showed abnormal findings. A large azygos vein placed posterior to the aorta. AZY, azygous vein; SVC, superior vena caval.


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