J Cardiovasc Ultrasound.  2011 Dec;19(4):211-215. 10.4250/jcu.2011.19.4.211.

Incidentally Detected Situs Ambiguous in Adults

• Kim JG
• Youn HJ
• Kim GH
• Park MH
• Hur J
• Yu JS
• Jung SY
• An SH

• KMID: 1980386
• DOI: http://doi.org/10.4250/jcu.2011.19.4.211

Abstract

Situs ambiguous is rare congenital anomaly in adults. In 2 adult patients who admitted for different cardiac problems, situs ambiguous with polysplenia was detected. A 42-year-old male admitted for radio frequent catheter ablation of atrial fibrillation, and he had left-sided inferior vena cava (IVC), hepatic segment of IVC interruption with hemiazygos continuation, multiple spleens and intestinal malrotation. And in a 52-year-old female case who was hospitalized due to infective endocarditis after implanting pacemaker for sick sinus syndrome, multiple spleens, left-sided stomach, bilateral liver with midline gallbladder, and left-sided IVC were found. Those findings were consistent with situs ambiguous with polysplenia, but their features were distinctive.

Keyword

Situs ambiguous; Polysplenia syndrome; Adult

MeSH Terms

Adult
Atrial Fibrillation
Catheter Ablation
Endocarditis
Female
Gallbladder
Heterotaxy Syndrome
Humans
Liver
Male
Middle Aged
Sick Sinus Syndrome
Spleen
Stomach
Vena Cava, Inferior

Figure

• 

  Fig. 1 Double the shadow of thoracic aorta (bold arrows) and widening state of superior mediastinum (sharp arrows) were shown in chest X-ray.

• 

  Fig. 2 (A) Coronary multidirectional computed tomography: There was an 1.7 cm sized, round, tubular structure which was paralleling with descending thoracic aorta (white arrows). (B-D) On abdomen computed tomography: (B) A dilated hemiazygos vein runs posterior to the descending aorta. (C) Multiple, well-defined round soft tissue densities were detected around the spleen (black stars). IVC was located at the left side of aorta, and hepatic segment of IVC was absent. (D) Left-sided colon (hollow arrow) and right-sided small bowels (bold arrow) indicated gastrointestinal malrotation. IVC: inferior vena cava.

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  Fig. 3 Venography of IVC through right femoral vein: Interruption of the thoracic IVC with hemiazygos continuation (arrows) along with aortic arch was confirmed. Enlarged hemiazygos vein drained into left brachiocephalic vein and then to superior vena cava. Infra-hepatic segment of IVC was intact. IVC: inferior vena cava.

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  Fig. 4 The chest X-ray of 52-year-old woman showed gastric air under the right side of diaphragm (arrows), and hepatic shadow in the left side abnormally.

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  Fig. 5 Liver dynamic computed tomography. A: There were midline symmetric liver (L) and multiple spleens (black stars) and stomach (S) are located at the right side of abdomen. B: Multiple sandy stones in midline gallbladder. Superior mesenteric vein was unusually located anterior to the superior mesenteric artery. C: The left sided inferior vena cava was crossing the aorta at the level of diaphragm and was drained to right atrium. D: Liver was observed in both sides relative to midline.

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