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EMGuideWire's Radiology Reading Room: Situs Anomalies

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Situs Anomalies
Michael A. Gibbs, MD, Lead Editor
Carolinas Medical Center Imaging Mastery Project
Dominic Nicacio, MD, An...

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EMGuideWire's Radiology Reading Room: Situs Anomalies

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The Department of Emergency Medicine at Carolinas Medical Center is passionate about education! Dr. Michael Gibbs is a world-renowned clinician and educator and has helped guide numerous young clinicians on the long path of Mastery of Emergency Medical Care. With his oversight, the EMGuideWire team aim to help augment our understanding of emergent imaging. You can follow along with the EMGuideWire.com team as they post these educational, self-guided radiology slides or you can also use this section to learn more in-depth about specific conditions and diseases. This Radiology Reading Room pertains to Situs Anomalies and is brought to you by Dominic Nicacio, MD, Andrew Yde, MD, Jorge Alegria, MD, and Laszlo Littmann, MD.

The Department of Emergency Medicine at Carolinas Medical Center is passionate about education! Dr. Michael Gibbs is a world-renowned clinician and educator and has helped guide numerous young clinicians on the long path of Mastery of Emergency Medical Care. With his oversight, the EMGuideWire team aim to help augment our understanding of emergent imaging. You can follow along with the EMGuideWire.com team as they post these educational, self-guided radiology slides or you can also use this section to learn more in-depth about specific conditions and diseases. This Radiology Reading Room pertains to Situs Anomalies and is brought to you by Dominic Nicacio, MD, Andrew Yde, MD, Jorge Alegria, MD, and Laszlo Littmann, MD.

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EMGuideWire's Radiology Reading Room: Situs Anomalies

  1. 1. Situs Anomalies Michael A. Gibbs, MD, Lead Editor Carolinas Medical Center Imaging Mastery Project Dominic Nicacio, MD, Andrew Yde, MD, Jorge Alegria, MD Laszlo Littmann, MD Departments of Emergency Medicine & Internal Medicine Sanger Heart & Vascular Institute Carolinas Medical Center
  2. 2. Visit Our Website www.EMGuidewire.com For A Complete Archive Of Chest X-Ray Presentations And Much More!
  3. 3. Airway Bones Cardiac Diaphragm Effusion Foreign body Gastric Hilum
  4. 4. It’s All About The Anatomy!
  5. 5. Embedded References Maldijan PD. Approach To Dextrocardia In Adults. American Journal Of Radiology. 2007; 188;S39- S49. Winter JE. Recognition of Anterior STEMI in Dextrocardia and the Importance of Right-Sided Chest Leads. Journal of The American College of Cardiology. 2020; 2(8):1222-1226. Gentile BA. Situs Inversus Totalis. New England Journal of Medicine. 2019; 380:e45. Celik AI. Acute Anterior Myocardial Infarction in a Patient with Dextrocardia and Situs Inversus. Journal of the American College of Cardiology. 2020; 2(8):1220-1221.
  6. 6. Situs Nomenclature Determined by the relationship between the atria and adjacent organs: • Situs solitus • Situs solitus with dextrocadia • Situs inversus with dextrocardia • Situs inversus with levocardia • Situs ambiguous
  7. 7. Situs Solitus • Normal anatomy Situs Solitus With Dextrocardia • Mirror image positioning of the heart only Situs Inversus With Dextrocardia [Totalis] • Mirror image positioning of all thoracoabdominal viscera Situs Inversus With Levocardia • Mirror image positioning of all abdominal viscera • Descending aorta on the right (concordant with situs inversus) Situs Ambiguous • Also called situs heterotaxy, any arrangement of organs between the spectrum of situs solitus (normal positioning) and situs inversus totalis
  8. 8. dextrocardia, the descending aorta, cardiac apex, and stomach are all on the right. In situs solitus with dextrocardia, the descending aorta and stomach are on the left (normal), but the cardiac apex is on the right. In situs inversus with levocardia, the descending aorta and stomach are on the right (situs inversus), but the cardiac apex is on the left. Figure 3-8 Schematic illustrations of the anatomic relationships of the descending aorta, left atrium, cardiac apex, and stomach in the four basic cardiac positions (normal and three malpositions). In situs solitus, the descending aorta, left atrium, cardiac apex, and stomach are all on the left. In situs inversus with dextrocardia, the descending aorta, left atrium, cardiac apex, and stomach are all on the right. In situs solitus with dextrocardia, the descending aorta, left atrium, and stomach are on the left (normal), but the cardiac apex is on the right. In situs inversus with levocardia, the descending aorta, left atrium, and stomach are on the right (situs inversus), but the cardiac apex is on the left. (RA = right atrium; LA = left atrium; RV = right
  9. 9. Situs Solitus With Dextrocardia
  10. 10. 3-11 X-ray from a 2-year-old girl in situs inversus with levocardia. The stomach (S) is on the right and the liver (L) is on the left, but the heart (apex) is to the left of mid midiaphragm is lower than the right hemidiaphragm because the cardiac apex is on the left. The descending thoracic aorta (DAo) is on the right (concordant for situs inversus of the ascending aorta (AAo) indicates a discordant d-bulboventricular loop. Situs Inversus And Levocardia: The stomach (S) is on the right and the liver (L) on the left. The left hemidiaphragm is lower because the cardiac apex is no the left. The descending thoracic aorta (DAo) is on the right (concordant with situs inversus, but the ascending aortic (AAo) is on the left.
  11. 11. Primary Ciliary Dyskinesia • Primary ciliary dyskinesia is an inherited disorder of abnormally functioning cilia • Abnormally functioning cilia of the epithelial cells necessary for correct thoracoabdominal orientation during embryogenesis • Approximately 25% of patients with situs inversus totalis have primary ciliary dyskinesia • Kartagener’s Syndrome defines a subset of primary ciliary dyskinesia patients with with classic triad: situs inversus totalis, sinusitis and bronchiectasis
  12. 12. Kartagener’s Syndrome
  13. 13. Epidemiology Dextrocardia Situs Inversus Totalis Incidence 1 in 12,000 1 in 10,000 Genetic Inheritance Inconclusive Autosomal recessive in patients with Primary Ciliary Dyskinesia Risk Factors  Males and females are affected equally  Maternal cocaine use is associated with an increased incidence Associated Conditions  Transposition of great vessels, double inlet ventricles, tricuspid atresia, polysplenia
  14. 14. Clinical Presentation Dextrocardia1,2,3:  Asymptomatic  Characteristic ECG findings Situs Inversus1,2,3:  Opposite sided abdominal complaints in acute hepatobiliary, gastric, and appendiceal pathology Kartagener’s syndrome1,2:  Chronic sinusitis and otitis media  Bronchiectasis  Infertility common in males 1Right-sided heart tones. 2Sequelea of any other associated congenital heart disease. 3Characteristic ECG findings.
  15. 15. Situs Anomalies Cases Studies From Carolinas Medical Center
  16. 16. Normal Position Situs Solitus Liver Diaphragm Higher On The Right Gastric Bubble Cardiac Apex Descending Aorta On The Left Spleen
  17. 17. 61-Year-Old At Cardiology Appointment.
  18. 18. Diaphragm Higher On The Left Situs Inversus With Dextrocardia Liver Gastric Bubble
  19. 19. LiverSpleen Abdominal Transposition Dextrocardia Situs Inversus Totalis
  20. 20. Healthy 33- Year-Old With Chest Pain.
  21. 21. Situs Inversus With Dextrocardia Gastric Bubbler Diaphragm Higher On The Left Liver
  22. 22. Healthy 42- Year-Old Has An Incidental Finding On Chest X-Ray
  23. 23. Situs Inversus With Dextrocardia Gastric Bubbler Liver
  24. 24. 65-Year-Old At His Cardiology Follow-Up Visit.
  25. 25. Diaphragm Higher On The Left Situs Inversus With Dextrocardia Gastric Bubbler Liver
  26. 26. Healthy 45- Year-Old Undergoes Preoperative Screening.
  27. 27. Situs Solitus With Dextrocardia Gastric Bubble Liver
  28. 28. 33-Year-Old With Congenitally Corrected Transposition Of The Great Vessels.
  29. 29. Situs Solitus With Dextrocardia Gastric Bubble Liver
  30. 30. Liver Situs Solitus And Dextrocardia
  31. 31. Classic ECG Findings • Inverted complexes in I, aVL • Upright complexes in aVR • Low voltage V3-V6 • No R wave progression • Right axis deviation • Normal EKG with lead reversal
  32. 32. If You Have Interesting Cases Of Situs Anomalies We Invite You To Send A Set Of Digital PDF Images And A Brief Clinical History To: michael.gibbs@atriumhealth.org Your De-Identified Case(s) Will Be Posted On Our Education Website And You And Your Institution Will Be Recognized!

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