A case of fetal heterotaxy syndrome.

Chon, Seung Joo; Kim, Suk Young; Lee, Soon Pyo; Hwang, Byoung Cheoul; Son, Dong Woo; Choi, Chang Hyu

doi:2011.54.10.630

Obstet Gynecol Sci > Volume 54(10); 2011 > Article

Case Report

Korean Journal of Obstetrics & Gynecology 2011;54(10):630-633.
Published online: October 1, 2011
DOI: https://doi.org/10.5468/KJOG.2011.54.10.630

A case of fetal heterotaxy syndrome.

Seung Joo Chon, Suk Young Kim, Soon Pyo Lee, Byoung Cheoul Hwang, Dong Woo Son, Chang Hyu Choi

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.

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.

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