Heterotaxy Syndrome
Diagnosis certain
Updates to Case Attributes
Body
was changed:
Heterotaxy syndrome (situs ambiguous) is a disturbance in the usual left-right distribution of the thoracoabdominal organs. Unlike situs inversus, heterotaxy does not entirely translate into a complete mirror image. This syndrome can be disturbed into one of two subsets:
1.) heterotaxy with isomerism of the right atrial appendages (asplenia syndrome)
2.) heterotaxy with isomerism of the left atrial appendages (polysplenia syndrome)
-<p>Heterotaxy syndrome (situs ambiguous) is a disturbance in the usual left-right distribution of the thoracoabdominal organs. Unlike situs inversus, heterotaxy does not entirely translate into a complete mirror image. This syndrome can be disturbed into one of two subsets:</p><p>1.) heterotaxy with isomerism of the right atrial appendages (asplenia syndrome)</p><p>2.) heterotaxy with isomerism of the left atrial appendages (polysplenia syndrome)</p><p> </p><p><a title="Heterotaxy syndrome" href="/articles/heterotaxy-syndrome">Heterotaxy syndrome</a></p>- +<p>Heterotaxy syndrome (situs ambiguous) is a disturbance in the usual left-right distribution of the thoracoabdominal organs. Unlike situs inversus, heterotaxy does not entirely translate into a complete mirror image. This syndrome can be disturbed into one of two subsets:</p><p>1.) heterotaxy with isomerism of the right atrial appendages (asplenia syndrome)</p><p>2.) heterotaxy with isomerism of the left atrial appendages (polysplenia syndrome)</p>
Presentation
was changed:
A 23-year-old female with a past medical history of orthotopic heart transplantation for complex congenital heart disease complicated by chronic antibody-mediated rejection.
Surgical history of distal pancreatectomy and right hemicolectomy.
References changed:
- Applegate K, Goske M, Pierce G, Murphy D. Situs Revisited: Imaging of the Heterotaxy Syndrome. Radiographics. 1999;19(4):837-52. <a href="https://doi.org/10.1148/radiographics.19.4.g99jl31837">doi:10.1148/radiographics.19.4.g99jl31837</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/10464794">Pubmed</a>
- 1. Applegate K, Goske M, Pierce G, Murphy D. Situs Revisited: Imaging of the Heterotaxy Syndrome. Radiographics. 1999;19(4):837-52; discussion 853-4. doi:10.1148/radiographics.19.4.g99jl31837 2. Ghosh S, Yarmish G, Godelman A, Haramati L, Spindola-Franco H. Anomalies of Visceroatrial Situs. AJR Am J Roentgenol. 2009;193(4):1107-17. doi:10.2214/AJR.09.2411 3. Applegate K, Anderson J, Klatte E. Intestinal Malrotation in Children: A Problem-Solving Approach to the Upper Gastrointestinal Series. Radiographics. 2006;26(5):1485-500. doi:10.1148/rg.265055167 4. Levine D, Barnewolt C, Mehta T, Trop I, Estroff J, Wong G. Fetal Thoracic Abnormalities: MR Imaging. Radiology. 2003;228(2):379-88. doi:10.1148/radiol.2282020604 5. Haramati LB, Glickstein JS, Issenberg HJ et-al. MR imaging and CT of vascular anomalies and connections in patients with congenital heart disease: significance in surgical planning. Radiographics. 22 (2): 337-47. 6. Donnelly L. Pediatric Imaging. (2009) ISBN: 9781416059073 7. Shapiro A, Davis S, Ferkol T et al. Laterality Defects Other Than Situs Inversus Totalis in Primary Ciliary Dyskinesia: Insights into Situs Ambiguus and Heterotaxy. Chest. 2014;146(5):1176-86. doi:10.1378/chest.13-1704 8. Kim S. Heterotaxy Syndrome. Korean Circ J. 2011;41(5):227-32. doi:10.4070/kcj.2011.41.5.227
Systems changed:
- Not Applicable
- Cardiac