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Case Reports
. 2022 Sep 27:16:11795468221116851.
doi: 10.1177/11795468221116851. eCollection 2022.

Heterotaxy Syndrome with Polysplenia, Fused Adrenal Glands, and Diabetes Mellitus

Affiliations
Case Reports

Heterotaxy Syndrome with Polysplenia, Fused Adrenal Glands, and Diabetes Mellitus

Abid M Sadiq et al. Clin Med Insights Cardiol. .

Abstract

Heterotaxy syndrome is a rare congenital heart disease with a disarrangement of the heart and abdominal organs. We present a young African female with features of heart failure, diffuse irregular cardiac murmurs, and palpable, tender epigastric mass. A chest and abdominal computed tomography (CT) identified heterotaxy syndrome with left isomerism and fused adrenal glands. This case highlights the feature of fused adrenal glands in a patient with polysplenia.

Keywords: Congenital heart disease; diabetes mellitus; fused adrenal glands; imaging; polysplenia; pulmonary arterial hypertension.

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Conflict of interest statement

Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
Electrocardiogram shows atrial fibrillation.
Figure 2.
Figure 2.
Echocardiogram shows (A) common atrium with a large atrioseptal defect. (B) Severe mitral regurgitation. (C) Severe tricuspid regurgitation.
Figure 3.
Figure 3.
Axial CT shows the common atrium with a large atrioseptal defect.
Figure 4.
Figure 4.
Three-dimensional virtual reconstruction shows a right-sided aortic arch, a large common atrium, and a left-sided superior vena cava.
Figure 5.
Figure 5.
Three-dimensional virtual reconstruction shows a right descending aorta and left pulmonary vein draining into the posterior cardiac atrium.
Figure 6.
Figure 6.
Axial CT shows a right aortic arch and left superior vena cava.
Figure 7.
Figure 7.
Three-dimensional virtual reconstruction shows a dominant right common carotid and a right subclavian artery arising from the aortic arch.
Figure 8.
Figure 8.
Coronal CT shows a midline liver, a left-sided inferior vena cava, and fused adrenal glands seen on either side of the descending aorta.
Figure 9.
Figure 9.
Coronal CT shows right-sided polysplenia (white circle under right hemidiaphragm).

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References

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